Guide Sjögren’s Syndrome: Practical Guidelines to Diagnosis and Therapy

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The best-described autoantibodies in pSS are the anti-Ro and anti-La antibodies, which are routinely identified as part of the ENA laboratory screen. These figures, however, depend on the classification criteria used, referral bias and access to labial gland biopsy. Patients present to primary care physicians, general physicians, ophthalmologists and dental practitioners many of whom lack specialist knowledge regarding the treatment of this patient cohort. This document aims to provide a pragmatic, practical guideline for the management of adults with pSS.

The target audience includes rheumatologists, general physicians, general practitioners, specialist nurses and other specialists e. The guideline should also be of relevance to specialist registrars in training and specialist nurses. The management of children with SS is not specifically covered by this guideline although the general management of children and teenagers with SS is similar to that for adults with special emphasis on the importance of good dental care and hygiene to preserve dental health.

SS is very rare in teenagers and there are only anecdotal reports worldwide of its occurrence in pre-pubertal children so we would recommend that children and teenagers presenting with symptoms suggestive of SS are referred to a specialist centre for evaluation and treatment. Where patients have secondary SS their systemic management should address the primary disease but the advice on topical management contained in these guidelines is applicable to sicca symptoms from any cause.

This guideline does not cover the detailed management of patients with lymphoma, who should be managed in conjunction with oncologists and haematologists. The multidisciplinary team involved in producing these guidelines was led by Dr Elizabeth Price, Consultant Rheumatologist, who chaired the team, undertook the systematic review and led the development of the guideline. Ms Katie L.

Sjögren's Syndrome Foundation - Clinical Practice Guidelines

Any conflicts of interest among members of the working party were fully declared. The declared conflicts of interest are included at the end of the article. Additional references were added through regular updates to the draft recommendations up to January Non-English language papers were excluded unless a translation was published.

We also excluded treatments not currently available in the UK unless there was a significant likelihood they would become available in the near future. Identified papers were reviewed, categorized and the level of evidence graded according to international criteria from Ia through to IV and A through to B [ 10 ].

See supplementary data Search history and supplementary table S1, available at Rheumatology online, for full details of search criteria and level of evidence definitions. The wording and content of the recommendations were subjected to a formal Delphi process [ 11 ] using online surveys to determine the eventual strength of agreement SOA for each recommendation. The Delphi method was developed in the s. It describes a process whereby a group of experts anonymously reply to questionnaires and subsequently receive feedback in the form of a statistical representation of the group response, after which the process repeats itself.

The goal is to reduce the range of responses and arrive at something closer to expert consensus. In our case each recommendation was rated on a score from 1 no agreement to 10 complete agreement.

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The results were collated and circulated to the group prior to the second round. Sicca symptoms can result from a variety of causes, some of which may be reversible if addressed. Drugs are a common cause of sicca and many different drug groups have been implicated. Potential causative agents include those with anti-cholinergic activity such as anti-depressants, anxiolytics and anti-psychotics; muscarinic anatagonists such as tamsulosin hydrochloride and ipratropium hydrochloride; anti-histamines; opiates; anti-hypertensives including beta-blockers and angiotensin converting enzyme ACE inhibitors; and proton pump inhibitors such as omeprazole.

Avoiding these entirely may not be practicable, but use should be minimized in the presence of sicca symptoms if possible. Sicca symptoms are commonly seen following head and neck radiotherapy and may respond to topical treatment and pilocarpine see below. Dehydration and acute anxiety may cause temporary sicca symptoms. Rarely, primary salivary gland pathology other than SS including sarcoidosis, IgG4 disease [ 12 ] and graft vs host disease [ 13 ] may be implicated.

Salivary gland aplasia and ductal atresia [ 14 ] are both rare causes of oral sicca and viral infections including hepatitis C and HIV can cause salivary gland disease with hypertrophy and sicca symptoms. Xerostomia can be a feature of oral dysaesthesias with no objective reduction in salivary flow rate. Oral dysaesthesia or burning mouth syndrome is a chronic pain condition currently classified as a neuropathic disorder.

Such patients may complain of a constellation of oral symptoms, including burning sensations, unusual taste as well as oral dryness, and their symptomatology is not uncommonly associated with anxiety states and clinical depression. There may also be a history of low back pain, FM and irritable bowel syndrome [ 15 ].

A syndrome comprising histological evidence of sialadenitis, nodular osteoarthritis and xerostomia has been described [ 16 ], but in view of the frequency of the individual clinical features and the demographics of the individuals with these conditions, clustering of these entities could also be expected to occur on a simple statistical basis. Ageing per se is not a cause of xerostomia.

Although there is a broadly linear reduction in the amount of functional acinar tissue within the salivary glands over time, this does not lead to clinically significant salivary gland hypofunction in normal subjects [ 17 ]. Xerostomia in the ageing population is more a function of intercurrent disease and medication rather than loss of secretory ability [ 18 ]. The guideline covers management of the eye and mouth manifestations, systemic dryness and systemic disease in general with comments on the management of pregnancy and lymphoma in patients with SS.

In general we would recommend initial symptomatic treatment of sicca manifestations combined, if appropriate, with systemic management. All patients with pSS and sicca from other causes should be offered symptomatic treatment of dryness. The sicca symptoms classically affect eyes and mouth but many patients also experience a chronic, dry cough due to drying of the mucosal surfaces and, in women, vaginal dryness.

In general treatment of sicca symptoms should aim to conserve, replace and stimulate secretions while reducing inflammation. Dry eyes may be classified as mild, moderate or severe based on the presence of both symptoms and signs [ 19 ]. In the past, staging of eye disease has not routinely been carried out by optomoterists and ophthalmologists but this is changing with routine staging being introduced for other eye diseases.

T able 1 Dry eye management summary. There is mucosal layer deficiency, aqueous deficiency and meibomian gland deficiency, all of which need to be addressed. Systemic medications that exacerbate dryness should be avoided if possible. These include those with anti-cholinergic activity psychotropics including antidepressants, anxiolytics and anti-psychotics , muscarinic antagonists e. Studies have shown that humidification of cabin air increases tear-film stability [ 20 ] in normal individuals and by extrapolation it is advised for dry eye.

Patients consistently report feeling more comfortable in a humidified environment. There is some low quality evidence that omega 7 derived from sea buckthorn oil provides some symptomatic relief of dry eye symptoms from both a small, open label, informal study [ 23 ] and a double-masked, randomized, parallel trial [ 24 ].

The evidence was felt to be of insufficient strength to include this as a recommendation within the final guideline. Adequate meibomian gland secretion is essential to prevent evaporative tear loss. There is evidence that it is useful to enhance meibomian gland secretion using either warm compresses or commercially available microwavable lid warming compresses [ 25 ]. Patients should be advised to perform lid hygiene if required using proprietary wipes available over the counter or with a weak solution of bicarbonate.

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A recent report by the American Academy of Ophthalmologists reviewed the evidence for the oral antibiotics doxycycline, minocycline and azithromycin in the management of ocular surface disease arising from disorders of the meibomian glands. They identified eight studies that documented an improvement in meibomian gland-related ocular surface disease after treatment with these agents, although side effects were common.

Only one study was a randomized, controlled trial. They concluded oral antibiotics may be an effective treatment for ocular surface disease that results from meibomian gland disease [ 28 ]. Expert advice in the UK is to consider doxycycline 50 mg od for a minimum of 3 months in patients with persistent meibomian gland inflammation and blepharitis not responding to topical management.

Minocycline is avoided because of the small risk of causing drug-induced lupus. The latter can be manufactured by opticians or purchased on-line. There is evidence supporting the early use of punctal plugs [ 33 , 34 ], which improve symptoms of dry eye significantly more than artificial tears alone. First line treatment should be with visible plugs. If this successfully improves symptoms or signs, and there is no evidence of excess watering or over flow of tears, then permanent punctal occlusion can be achieved with punctal cautery.

Intracanalicular plugs are effective but increase the risk of granuloma and infection [ 35 , 36 ] and are not visible on examination. Referral to an ophthalmologist is advised and it is important to remember that plugs are available in different sizes and the puncta should ideally be measured with a calliper prior to selection and insertion of an appropriately sized plug. Improperly sized plugs may fall out, with accompanying deterioration in symptoms; alternatively loose plugs will allow tear flow, or cause irritation.

Treatment should begin with conservation of tears by humidification and avoidance of systemic medication that exacerbates dryness. Patients should be advised to stimulate meibomian gland secretion daily using either warm compresses or commercially available microwavable lid warming compresses or other devices and perform lid hygiene if required using proprietary wipes available over the counter or with a weak solution of bicarbonate. In patients with persistent meibomian gland inflammation and blepharitis, treatment with doxycycline 50 mg od for a minimum of 3 months may be effective.

Early referral to an ophthalmologist for review and consideration of insertion of punctal plugs or cauterization of puncta is recommended. Liposomal sprays available over the counter but not currently FP10 prescribable reduce evaporative tear loss and replace the meibomian gland layer and have been shown to significantly reduce sicca symptoms [ 37 ]. To replace the aqueous tear film start with simple lubricating drops using the most cost-effective options.

In general start with low viscosity simple drops such as hypromellose and escalate as symptoms dictate. Given the severity of dry eye disease in primary SS, it is unlikely, however, that simple drops will be sufficient to control the disease. When prescribing take into account the bottle or device used to apply the drops. In particular consider ease of application and expiry dates. See advice below and in accompanying Table 2 for preparations. T able 2 Dry eye topical therapies.

A selection of ocular lubricants available in preserved and non-preserved forms. Most are available both over the counter and on prescription, some are prescription only and others are over the counter only. Multi-use ophthalmic preparations rapidly become colonized with bacteria and are associated with high rates of pathogenic organisms on conjunctival culture [ 38 ].

As a result, the majority of multi-dose preparations contain a preservative to limit bacterial, fungal and amoebic ocular infections and prolong shelf life by preventing biodegradation. High concentrations of certain preservatives may damage and irritate ocular tissue and this effect is enhanced in those with ocular surface disease. Benzalkonium chloride is the most frequently used preservative in topical ophthalmic preparations and its epithelial toxic effects are well established [ 39 ].

Some eye drops are available as single-use preservative-free preparations. Although convenient these are often costly and require good manual dexterity to use. Increasingly eye drops are available in multi-use bottles with preservative systems that biodegrade on contact either with light or with the ocular surface. Other preservative-free multidose systems use a pump or vacuum system preventing regurgitation into the bottle and obviating the need for preservatives altogether.

Autologous or allogeneic serum eye drops are the only available nutritional tear substitutes and are effective and well tolerated by patients [ 40 , 41 ]. However, they are very expensive to produce and are only available from commissioned specialist centres. Recommend liposomal sprays to reduce evaporative tear loss and replace the meibomian gland layer. Start with simple lubricating drops using the most cost-effective options see Table 2 for preparations. Patients with severe dry eye, not responding to conventional treatment may be referred to specialist centres for consideration of autologous or allogeneic serum eye drops.

There is evidence supporting the use of topical anti-inflammatories under ophthalmic supervision in patients with persistent corneal inflammation. Low dose steroid-containing eye drops are recommended for short term use and have been shown to be both safe and effective [ 42 ]. Longer term use of topical steroids has been associated with an increased risk of glaucoma and cataract and should be avoided [ 43 ]. In general the use of topical steroids should be limited to patients under supervision by an ophthalmologist.

For longer term use ciclosporin-containing eye drops have been shown to reduce subjective symptoms and improve objective signs of inflammation in patients with both primary and secondary SS [ 44—46 ]. Ciclosporin 0. Topical NSAIDs such as diclofenac and indomethacin have been shown to improve ocular discomfort but reduce corneal sensitivity and may predispose to further corneal damage and should therefore be used only with caution in patients with SS [ 48 , 49 ].

Low dose steroid-containing eye drops are recommended for short term use under ophthalmic supervision only. Ciclosporin-containing eye drops or ointments may be used under ophthalmic supervision in patients with evidence of chronic inflammation. Topical NSAIDs such as diclofenac and indomethacin should be used with caution under ophthalmic supervision only. Oral pilocarpine treatment can improve ocular dryness and tear film break-up time and increase goblet cell density thus improving the mucous layer [ 50—54 ].

Symptomatic and objective improvements are seen in more than a third of patients, although side effects headache, increased sweating may limit its use in some. The recommended starting dose is 5 mg once daily, escalating at weekly intervals as tolerated to 5 mg qds with meals and at bedtime but if response is insufficient, the dose can be increased to 30 mg daily, if tolerated well; discontinue if no improvement after 2—3 months.

Contra-indications include uncontrolled asthma and chronic obstructive pulmonary disease, uncontrolled cardiorenal disease and acute iritis. Common side effects include dyspepsia, diarrhoea, abdominal pain, flushing and increased urinary frequency. There is evidence from an observational study that it is both safe and effective in children and young people as young as 9 years of age [ 55 ].

In patients intolerant of pilocarpine tablets, using topical ocular drops orally allows delivery of a smaller dose of drug and may be better tolerated. A trial of pilocarpine 5 mg once daily increasing stepwise to 5 mg qds is recommended for patients with significant sicca symptoms and no contraindications to its use.

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Some patients with severe dry eye develop an associated blepharospasm. There are small case series describing a good response to botulinum toxin injections in these circumstances [ 59 , 60 ]. For severe dry eye bandage scleral contact lenses, designed to protect the surface and create an optimized environment for healing, may be indicated.

These lenses vault over the corneal surface and rest on the sclera. They are rigid and gas-permeable and require specialist fitting and manufacture. They are only available in specialist centres for severe corneal disease refractory to other treatment measures. A number of studies have shown significant decreases in corneal staining with scleral contact lens wear with significant improvements in dry eye symptoms and quality of life [ 61—63 ].

Corneal grafting or amniotic membrane overlay may occasionally be indicated for cases of significant corneal ulceration and corneal melt [ 64 ]. Neuropathic pain when dry eye symptoms outweigh clinical findings is a recognized entity. Neuropathic pain strategies should be considered for these patients [ 65 ]. Mucolytic eye drops should be prescribed for patients with mucous threads or ocular surface filaments. Patients with severe dry eye and associated blepharospasm may benefit from referral to a specialist centre for consideration of botulinum toxin treatment.

Patients with severe dry eye and corneal ulceration, not responding to conventional treatment, may be referred to specialist centres for consideration of bandage contact lenses or amniotic membrane grafting or corneal grafting for perforations. Dry mouth xerostomia is a hallmark of pSS and the majority of patients suffer from both reduced salivary flow rate and increased viscosity of saliva due to relative deficiency of the aqueous component, which may compromise oral health.

Complications of long-term xerostomia include oral discomfort and soreness and a burning sensation in the mouth. An increased rate of dental caries, excessive tooth wear and premature tooth loss are often observed. Patients struggle to achieve a high standard of oral hygiene and may suffer from halitosis. Patients tolerate dentures poorly and often complain of difficulty retaining them in the mouth with sore, ulcerated gums as a consequence of rubbing.

Many patients suffer recurrent infections including parotitis, intraoral candidiasis and angular cheilitis. Dysarthria, dysphagia and dysgeusia distorted sense of taste are seen in established disease. Effective management of the dry mouth is important for the maintenance of oral health and to improve oral health-related quality of life as well as general health and quality of life.

Many patients with pSS anecdotally report improvement in symptoms following humidification of room air and patients frequently report worsening of symptoms in dry and air-conditioned environments. Patients with pSS have higher rates of the cariogenic bacterium Streptococcus mutans , dental caries and dental loss compared with controls despite apparently good dental care [ 68 , 69 ]. Dental caries is considered to be a diet-mediated disease, as sugars are essential in the caries process. This has been confirmed in several large-population-based studies that have shown a significant association between total sugar intake and the development of dental decay over time in a normal population.

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Twice daily use of fluoride toothpaste reduced but did not eliminate the association between diet and dental decay in these studies [ 70—72 ]. Given their enhanced risk of dental decay patients with pSS should be advised to practice excellent oral hygiene and avoid sweetened food and drink on a regular basis.

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The optimum concentration of fluoride in standard toothpaste has been calculated assuming a normal salivary flow rate and taking into consideration the availability of fluoride in normal saliva. Concentrations of fluoride in standard over-the-counter toothpastes vary from to parts per million ppm and occasionally higher.

Prescription toothpastes contain fluoride concentrations of between and ppm. Dry mouth patients will need a higher concentration of fluoride in toothpaste to compensate for the lack of salivary fluoride. A number of studies [ 73—75 ] and a Cochrane review [ 76 ] have concluded that caries protection is increased with higher fluoride concentrations. A systematic review of the literature and a Delphi consensus exercise by a national panel of experts in North America concluded that there was good evidence that a prescription strength, home-use, fluoride gel or paste should be used in all patients with pSS [ 77 ].

Acidic products and those containing sugar should not be prescribed for dentate patients since maintaining a neutral oral pH environment may reduce the development of dental caries [ 78 ]. There is evidence that in addition to the total sugar content of the diet, frequency of sugar intake is implicated in dental decay [ 77 ].

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In light of this we recommend that patients with pSS avoid food and drinks other than plain water between meals and from 1 h before bedtime and all through the night. Patients should be advised to brush their teeth at least twice daily but not immediately after eating including before bed at night using a pea sized amount of high fluoride toothpaste and use fluoride-containing tooth mousse or gel on teeth twice daily. They should avoid alcohol-containing mouth washes, sugary or fizzy drinks and frequent snacking [ 77 ]. Chlorhexidine is an effective antiseptic that inhibits plaque formation on teeth and is useful for controlling gingivitis [ 79 ].

Many oral specialists and dentists recommend an alcohol-free chlorhexidine mouth wash in pSS patients as preventative treatment for gum disease. It can be used twice daily for a maximum of 2 weeks every 3 months but over use can lead to staining of teeth. Patients should be advised to humidify the environment. Patients should be advised to practice excellent oral hygiene, limit sugar intake and avoid food and drinks other than plain water between meals and from 1 h before bedtime and all through the night.

Aim to avoid acidic products and those containing sugar when prescribing for dentate patients. Patients should be advised to brush teeth at least twice daily but not immediately after eating including before bed at night using a pea sized amount of high fluoride toothpaste and use fluoride-containing oral mousse or gel on teeth twice daily. Alcohol-free chlorhexidine mouth wash used twice daily for a maximum of 2 weeks every 3 months can help prevent gum disease but over use can lead to staining of teeth.

It is common practice to manage mild symptoms by recommending that patients sip water, suck ice or chew sugar-free gum [ 77 , 80 ]. More severe dryness can be managed with commercially available topical and systemic treatments for dry mouth. Saliva substitutes provide symptomatic momentary relief of oral dryness. A Cochrane review of the effectiveness of topical treatments for dry mouth from any cause, including parallel and crossover randomized controlled trials of lozenges, sprays, mouth rinses, gels, oils, chewing gum and toothpastes did not find strong evidence for their effectiveness [ 81 , 82 ], but in the experience of the working group patients report increased oral comfort from their use.

The ideal preparation will have neutral pH and contain fluoride and other electrolytes, mimicking the composition of natural saliva. Saliva substitutes are available commercially in the form of oral sprays, gels and rinses. Most are freely available over the counter and some are prescription only. The choice will depend upon patient preference and local availability.

Fluoride-containing, neutral pH preparations should be prescribed for dentate patients see Supplementary table S2, available at Rheumatology online, for products available in the UK. There is limited evidence that an oxygenated glycerol triester saliva substitute spray is more effective than a water-based electrolyte spray in older patients with dry mouth [ 83 ]. There is evidence that polyvinylpyrrolidone and sodium hyaluronate oral gel Gelclair may be helpful in patients with recurrent oral ulceration post-chemotherapy but no published studies of its effectiveness in SS [ 85 ].

A list of commercially available oral preparations is provided for reference in Supplementary table S2, available at Rheumatology online. Patients should be advised to use a neutral-pH fluoride-containing mouth wash, gel or spray as required for symptomatic relief. Although no study directly links improved salivary flow to caries prevention, expert opinion acknowledges that increasing saliva flow is likely to reduce caries incidence in the longer term. Chewing sugar-free gum increases saliva production and there is some evidence that in addition to its value as a non-sugar sweetener xylitol may have a role in caries prevention in its own right [ 86 , 87 ].

A recent Cochrane review of the evidence for xylitol concluded that over 2. Many patients chew or suck sugar-free sweets and report benefit, but there is no published evidence supporting this. Certain foodstuffs may be more effective at stimulating saliva than others, but acidic products and those containing sugar should be avoided because of their cariogenic potential.

A small cross-over study in normal volunteers assessed the efficacy of yogurt and lemon juice as salivary stimulants and found that both were effective but yogurt more so than lemon juice compared with baseline, leading them to suggest that yogurt is a potential candidate for the treatment of dry mouth and warrants further study [ 89 ]. Some commercially available salivary stimulants are available in the UK. There is little convincing evidence of efficacy and conflicting results from the few reported studies [ 90 , 91 ].

A small single-blinded, open-label, cross-over clinical study of a branded dry mouth product Xerostom containing olive oil, betaine and xylitol in a population with polypharmacy-induced xerostomia resulted in increased unstimulated whole salivary flow rates, reduced complaints of xerostomia and improved xerostomia-associated quality of life. No clinically significant adverse events were observed [ 92 ]. The preparation is non-acidic and contains both fluoride and calcium so may be suitable for patients with SS, although there are no published studies in this patient population.

Anhydrous crystalline maltose is available over the counter or on-line and has been shown to increase salivary flow rates with minimal adverse effects in small, observational studies [ 93 ] but is not available on prescription. There is some experimental evidence for both acupuncture and mild electrical stimulation improving salivary flow rates. Published studies are generally small, usually observational and are not specifically looking at patients with SS [ 81 ]. There were adverse effects reported with the use of acupuncture, but these were mild and of short duration.

There were no reported adverse effects from electrostimulation, with some evidence for improved salivary flow. Further larger studies are planned but neither is currently routinely available in the UK for the treatment of sicca [ 94 ]. A gel-releasing device reservoir worn in the mouth has been trialled and may be effective in some patients [ 95 ], but more research is needed.

Patients should be advised to chew xylitol-containing sugar-free gum. It acts as an agonist on muscarinic-cholinergic receptors to stimulate secretion. It is effective in both primary and secondary SS. Evidence suggests that at least half of patients respond although it can take up to 12 weeks to see a therapeutic benefit. In long term use levels of candida colonization reduce [ 99 ]. Side effects are common and dose dependent.

They include sweating, palpitations and flushing. Cevimeline is another muscarinic agonist that stimulates fluid secretion generally [ , ] but has higher affinity for muscarinic receptors located on lacrimal and salivary gland epithelium and may be associated with fewer side effects compared with pilocarpine [ ]. However, this drug is not currently licensed for use in the UK. Patients may have visible white plaques simple infection or erythematous infection, when they present with a red, raw tongue or oral cavity. Clinical experience suggests that whereas topical agents such as nystatin may be adequate to treat simple infection, oral fluconazole is required to treat erythematous infection.

The presence of dentures creates an environment where candida may become persistent. Angular cheilits, where patients present with dry, fissured erythematous lesions in the corner of their mouths, is another manifestation of candidal infection in SS. It can be effectively treated with miconazole topically for 2 weeks, using a clean cotton bud to apply to each side to prevent cross-contamination and persistence of infection [ ].

In simple candida infection visible white plaques treat with oral nystatin liquid 1 ml five times daily for 7 days. Consider repeating the treatment for 1 week every 8 weeks if frequent recurrence. If the patient has erythematous infection manifesting as a red, raw tongue or oral cavity and commonly seen in denture wearers, use fluconazole 50 mg od for 10 days. Treat angular cheilitis with miconazole topically for 2 weeks, using a clean cotton bud to apply to each side to prevent cross-contamination and persistence of infection.

Some patients with pSS have recurrent or persistent salivary gland enlargement. This may occur during disease activity flares or may reflect duct obstruction due to strictures, stones or inspissated secretions. Parotid sialography is a very specific diagnostic test for pSS [ ] that involves injection of contrast materials into the salivary ducts and is associated with a small risk of infection, ductal perforation and allergic reactions. Intraductal injection of contrast can assess the ductal system and exclude strictures [ ]. Interventional sialoendoscopy has been reported to be useful in patients with SS with chronic obstructive sialadenitis unresponsive to medical therapy.

Under local anaesthesia the orifice of the salivary gland is intubated and the scope advanced, with continuous saline lavage. This allows obstructing plaques to be washed out and any strictures dilated. However, sialoendoscopy is not widely available and no large scale or long term studies have been published. In contrast salivary gland US is non-invasive, well tolerated by patients and easily repeatable.

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US allows assessment of the architecture of the major salivary glands and, used in conjunction with Doppler signals, it may help differentiate between benign and malignant lesions. Baseline ultrasound is recommended in patients with salivary gland swelling to look for active inflammation, infection and stones. Consider repeating the US if there is a history of new persistent or recurrent salivary gland swelling to monitor for disease progression and development of complications [ ].

If there is acute inflammation in the absence of infection and stones then a short course of oral prednisolone or an intra-muscular injection of depomedrone typically mg will often settle the glandular swelling promptly anecdotal evidence. There is anecdotal evidence that massaging the glands can help reduce discomfort in chronically inflamed glands. Consider salivary gland US to look for active inflammation, infection and stones.

If acute inflammation in the absence of infection and stones then a short course of oral prednisolone or an intra-muscular injection of depomedrone will often settle the glandular swelling. Massaging the glands can help reduce discomfort in chronically inflamed glands. Many patients report a chronic dry cough due to drying of mucosal surfaces. Humidification of inspired air is reported to help by some patients [ ]. Pilocarpine improves secretions in general and anecdotally reduces the frequency and severity of dry cough. A mucolytic agent i. There is anecdotal evidence it helps in patients with SS and an associated chronic, dry cough.

It should be remembered that chronic dry persistent cough may be a sign of ILD and should prompt appropriate investigations. Females with SS frequently complain of vaginal dryness [ ]. Pilocarpine may help this by non-specifically stimulating secretions. There is evidence supporting the use of non-hormonal moisturisers in the treatment of vaginal dryness from any cause [ — ]. Vaginal moisturizers applied on a regular basis have an efficacy equivalent to local hormone replacement for the treatment of local urogenital symptoms such as vaginal itching, irritation and dyspareunia [ ].

Patients with SS have both vaginal dryness and atrophy of the genital skin [ ]. There is evidence supporting the use of topical oestrogen-containing products in patients with post-menopausal vaginal atrophy and dryness [ ]. Creams and pessaries are equally effective and although systemic absorption can occur with local preparations, there is no current evidence of an increased risk of endometrial thickening [ ]. The use of local hormone replacement therapy can reduce the frequency of recurrent urinary tract infections in post-menopausal women [ ].

A trial of pilocarpine 5 mg once daily increasing stepwise to 5 mg qds is recommended for all patients with significant systemic dryness and no contraindications to its use.

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Patients with vaginal dryness should be advised to use a non-hormonal vaginal moisturiser with or without a topical oestrogen-containing product. The most commonly involved organs are joints, lungs, skin and peripheral nerves [ 3 ]. Other systemic features may include autoimmune liver disease, renal involvement, scLE, ITP, myositis, monoclonal gammopathy of uncertain significance and lymphoma. A recent systematic review evaluated the evidence for non-pharmacological interventions for pSS and found that much of the evidence was inconclusive [ ].

The available studies suggest that patients with SS benefit from moderate to high intensity exercise with improvements in aerobic capacity, fatigue scores, physical functioning and depression [ ]. Lifestyle management strategies may benefit SS patients if fatigue is significantly affecting their ability to perform daily activities. Strategies recommended by NICE guidance for fatigue include sleep management, activity management, relaxation techniques, cognitive behavioural therapy and graded exercise therapy.

See relevant NICE guidance on management of fatigue in adults. Arthritis Research UK www. Regular exercise and, where appropriate, a graded exercise programme are recommended for patients with SS presenting with fatigue. Provide written patient information and details of appropriate support groups and on-line resources. Early and aggressive immunomodulation is recommended for RA and increasingly for other autoimmune diseases but not well established for SS. An evidence base is lacking and patients with SS often tolerate these drugs poorly.

There is, however, increasing insight into the pathogenesis of SS and interest in its therapeutic management with an increase in the use of conventional disease modifying drugs and research interest in the development of targeted biological agents. The guideline working group considered the evidence supporting the use of corticosteroids, disease modifying drugs, biologics and other drugs alongside their own experience.

Usual interstitial pneumonitis and non-specific interstitial pneumonitis are the most commonly reported. In general systemic steroids are recommended as first line treatment, then subsequently various immunosuppressives, including CYC and AZA, rituximab and anti-malarials depending on steroid responsiveness [ — ].

It does not usually respond to treatment with steroids and in general is treated as for trigeminal neuralgia of unknown cause. It is associated with vascultis and may respond to treatment with steroids and CYC [ ]. Autonomic neuropathy is common and correlates with patient reported outcomes [ ]. It may respond to simple, symptomatic treatment. SS myelopathy can mimic multiple sclerosis. Patients may present with paraplegia, sensory changes and bladder dysfunction.

Steroids and CYC may be helpful [ ]. Correlation with serological abnormalities and clinical symptoms is poor, however. This patient group benefits from aggressive and ongoing immunosuppression [ ]. In those patients with tubulointerstitial nephritis there is a mononuclear lymphocytic infiltrate on renal biopsy, and treatment with mycophenolate and steroids improves the renal function [ ]. Prednisolone has been shown to help systemic features including lung disease [ — ] cytopaenias [ ], and, in combination with CYC or chlorambucil, neurological involvement [ , ].

Small studies of low dose prednisolone 5—7. There are no controlled trials of pulsed i. Corticosteroids are not recommended for the routine treatment of pSS although intermittent short courses of oral or intramuscular steroid are effective for systemic flares of disease and steroids may be used for significant organ manifestations with or without additional immunosuppressive treatment. Low dose oral prednisolone is effective in treating persistent constitutional symptoms in patients who have failed to respond to HCQ or other immunosuppresants.

A number of studies [ — ] suggest modest improvements in ocular and oral dryness, lowering of ESR and immunoglobulin levels, together with reductions in fatigue levels and joint pain in patients treated with HCQ. The study was extended to 48 weeks with all patients being prescribed HCQ between weeks 24 and Although there was a suggestion of clinical and serological improvement in the cohort taking a full 12 months of HCQ, in the open-label extension this did not reach statistical significance [ ].

A meta-analysis of six earlier trials involving female patients failed to identify a clear benefit of antimalarials but commented that the available evidence was of poor quality and called for more and better quality research [ ]. All members of the guideline working group recommended a therapeutic trial of HCQ treatment in patients with evidence of systemic disease starting at mg once daily decreasing after 6 weeks to mg once daily with monitoring as per BSR guidelines.

If no response after 12 months of treatment consider stopping. AZA has been reported as helpful in case reports for systemic complications such as lung disease [ ], myelopathy [ ] and cytopaenias [ ], but a double blind placebo controlled trial in a small cohort of patients with uncomplicated disease suggested that it did not have a routine role in treatment and was associated with a high frequency of side effects [ ].

Discontinuation rates of AZA due to non-respiratory side effects may be higher than for mycophenolate although efficacy is similar in patients with ILD [ ]. MTX is the drug of choice for patients with significant inflammatory arthritis [ ]. An open label, pilot study of weekly MTX in 17 patients showed improvement in sicca symptoms, parotid swelling, dry cough and purpura but no improvement in objective parameters of dry eyes and mouth [ ]. MTX may be useful for those patients with an associated inflammatory arthritis but there is not enough evidence for its routine use as a disease-modifying drug for those with sicca symptoms alone.

A single centre, open-label pilot trial of mycophenolate in 11 patients reported subjective improvement of ocular dryness and reduction in artificial tear use, but objective evidence of significant glandular improvement in only two patients with short disease duration. There was significant reduction in hypergammaglobulinaemia and RF levels and increase in complements and white cell levels [ ].

Mycophenolate has been show to stabilize scleroderma associated ILD in a randomized controlled trial [ ]. The use of mycophenolate may be considered in patients with systemic complications such as cytopaenias or lung disease. There are no controlled trials of CYC in SS and in general its potential toxicity would preclude routine use. The use of CYC usually in combination with steroids may be considered in patients with organ threatening systemic complications such as CNS, renal or lung disease.

Two phase II open label pilot studies found very modest benefits and multiple side effects [ , ]. LEF is not routinely recommended for pSS. A prospective open label study found very modest benefits and multiple side effects [ ]. There is accumulating evidence from published case studies and case series supporting the use of rituximab in patients with active, systemic pSS.

A number of open label studies and randomized controlled trials have looked at the effect of rituximab on the fatigue and sicca manifestations see Supplementary table S3, available at Rheumatology online, for a summary. An open label study of 12 patients [ ] observed modest improvements in patient reported fatigue and oral dryness at 26 weeks following a single treatment course of rituximab. The TEARS Tolerance and Efficacy of Rituximab in primary Sjogren's syndrome study [ ] found no significant differences between treatment and placebo arms in the primary end point an improvement of at least 30 mm in 2 of 4 VAS scores at week 24 following a single treatment course of rituximab.

However, there was a significant improvement in the fatigue VAS scores in the treatment group at 6 and 16 weeks. Although preliminary analysis has shown no consistent benefit on fatigue, dryness and salivary flow, further analysis of data is being conducted. A recent study has suggested that patients with higher levels of B cell infiltration within the parotid glands are more likely to respond to rituximab, suggesting that we may in future be able to target therapy more effectively [ ].

There is evidence that B cell activating factor is upregulated after rituximab treatment [ ] and this may explain the observation in the TEARS study that patients had improved at 4 months but deteriorated again by 6 months [ ]. The dose, duration between doses and treatment response in the smaller studies and case series are listed in Supplementary table S3, available at Rheumatology online, but in the TEARS study [ ] patients received just two 1 g pulses of rituximab 2 weeks apart at baseline, whereas in the TRACTISS study [ ] this was repeated at 6 months.

In conclusion there is currently insufficient evidence to recommend routine use of rituximab in those with predominantly fatigue and sicca manifestations but good evidence for its use in systemic complications and lymphoma. Use of Rituximab in pSS is not currently NICE approved and is not routinely funded for the treatment of SS with the exception of those patients presenting with lymphoma. Rituximab is recommended for specialist use in patients with significant systemic manifestations refractory to treatment with steroids and other immunosuppressives.

It has a role in those with lymphoma, ITP, vasculitic neuropathy and cryoglobulinaemias. Belimumab is a human mAb that inhibits B cell activating factor. Belimumab is not currently recommended although it merits further study. Abatacept was well tolerated in one open label pilot study and fatigue and health related quality of life improved significantly over a week treatment period [ ]. Abatacept is not currently recommended although it merits further study. We describe a year-old woman with hypertension. Several weeks earlier, she experienced the onset of progressive asthenia, fatigue, and dyspnea as well as chest pain of nonspecific characteristics, symptomatic hypotension, and syncope; she did not report fever.

At the time of admission, she presented systolic blood pressure of 90 mm Hg, electrocardiogram with sinus tachycardia, right bundle-branch block plus left anterior bundle-branch block, normal PR interval, and cardiomegaly and congestion on chest x-ray. The basic analyses obtained the following: creatinine 1. All blood cultures and serology tests were negative. No alterations were observed on ventilation-perfusion scintigraphy or chest and abdominal CT scan, and Holter monitoring showed no arrhythmias.

Corticoid therapy was initiated, with rapid clinical improvement. C-reactive protein had dropped to 1. The etiologic mechanism of this myocarditis has been related to a possible leukocytoclastic vasculitis.. We consider that, in cases of severe dysfunction of uncertain etiology, systemic inflammatory activity and a specific autoimmune etiology should always be considered, as the most appropriate treatment may be simple and effective.. Descargar PDF. Texto completo. Treatment with corticoids and in more severe cases, azathioprine and cyclophosphamide is ineffective in sicca syndrome, but effective for the systemic manifestations.

The etiologic mechanism of this myocarditis has been related to a possible leukocytoclastic vasculitis. We consider that, in cases of severe dysfunction of uncertain etiology, systemic inflammatory activity and a specific autoimmune etiology should always be considered, as the most appropriate treatment may be simple and effective. Arch Inst Cardiol Mex, 67 , pp. Int J Cardiol, 70 , pp.

Arthritis Rheum, 39 , pp. Scand J Rheumatol, 34 , pp. Ann Rheum Dis, 57 , pp.