Approach

Treatment for Sjogren syndrome is largely symptomatic and the prevention and treatment of complications such as corneal ulceration and secondary infection, accelerated dental caries, tooth breakage/loss, and oral candidiasis. Explanation of symptoms to the patient is important, including reassurance that lifespan is generally not shortened.[85]

Glandular symptoms

Alleviation of dry eye symptoms is the primary focus of treatment. Options include:[12][86][87]

  • Artificial tear substitutes

  • Ciclosporin eye drops

  • Intermittent corticosteroid eye drops

  • Cholinergic drugs that stimulate tear secretion (e.g., cevimeline, pilocarpine). These drugs are effective in the treatment of sicca symptoms in patients with Sjogren syndrome.

It is important to avoid anticholinergics, antihistamines, and diuretics, which can exacerbate eye dryness. Spectacle eye shields are a recommended adjunct to help maintain a humid environment, and patients should take regular breaks while reading.[87][88][89]​​​

Intermittent corticosteroid eye drops may be helpful as an adjunct therapy for patients being treated with ciclosporin eye drops to reduce the time to symptom relief, or for patients with ocular inflammation who have not responded to artificial tears/lubricants and ciclosporin eye drops.[90][91][92]

Punctal plugs or permanent punctal occlusion may be an adjunct to other treatments when artificial tears and eye drops are insufficient to provide relief for dry eyes.[87]​ Significant improvements in Schirmer's test, staining with Rose Bengal/fluorescein, and tear break-up time have been described in 19 patients with primary Sjogren syndrome (pSS) at 24 months, after thermal punctal occlusion.[93]

Treatment for dry mouth has been mainly palliative, although cevimeline and pilocarpine are both effective in the treatment of dry mouth from Sjogren syndrome.

  • Simple precautions to minimise water loss from secretions by evaporation (use of humidifiers; moisturising creams or petroleum jelly on lips to prevent cracking and dryness).

  • Salivary substitutes for improving lubrication and hydration of oral tissues. Gels (fluoride gels), saliva-stimulating lozenges or sugar-free chewing gums, mouthwashes, prescription-strength toothpastes, and oral rinses have been used for this purpose.[36]

  • Temporary comfort can be obtained by sipping water and other sugar-free liquids.

  • Medications such as antidepressants, those used for Alzheimer's disease, antipsychotics, benzodiazepines, diuretics, stimulants, and bronchodilators can cause dry mouth, and should be avoided if possible.

Evidence of benefit for sicca symptoms is sparse in patients with Sjogren syndrome for other treatments, such as oral corticosteroids, hydroxychloroquine, methotrexate, ciclosporin, and rituximab.[94][95][96][97][98]​​[99][100][101][102][103][104][105][106][107][108]

Extraglandular manifestations

In the absence of large randomised controlled studies, the treatment of extraglandular manifestations are mainly case-dependent and empirical.

Fatigue

Fatigue affects about 50% of patients. There is no specific treatment. A consensus recommendation suggests that hydroxychloroquine may be used to treat fatigue, but there are no randomised clinical trials supporting this intervention.[109]​ Potential use of hydroxychloroquine should only be considered after comprehensive evaluation of disease activity, sicca manifestations, and subjective variables, and should be individualised according to the clinical context.[109] Concomitant hypothyroidism, fibromyalgia, lymphoma, or underlying depression should be considered, and treated if present. See Primary hypothyroidism, Fibromyalgia, Non-Hodgkin's lymphoma, Hodgkin's lymphoma, and Depression in adults.

Musculoskeletal manifestations

Simple analgesics such as paracetamol are a safer alternative to non-steroidal anti-inflammatory drugs (NSAIDs) and, if effective, should be used in preference. The use of NSAIDs for arthralgia/myalgia/arthritis in this syndrome is not evidence based.

Hydroxychloroquine is recommended as a first-line disease-modifying anti-rheumatic drug (DMARD) for inflammatory musculoskeletal pain in patients with pSS.[109] If the patient does not respond to hydroxychloroquine alone they should be switched to methotrexate alone. If both monotherapies are ineffective, combination treatment with hydroxychloroquine plus methotrexate can be considered.[109]​

A short course (1 month or less) of an oral corticosteroid is an option for patients who do not respond to hydroxychloroquine plus methotrexate.[109] A longer course of corticosteroids may be effective, but a corticosteroid-sparing agent should be added as soon as possible.[109]​

If the patient does not respond to initial treatments, leflunomide, sulfasalazine, azathioprine, or ciclosporin can be considered.[109] However, the recommendations for these treatments are based on weaker evidence, and choice of treatment should be guided by the physician's experience and the needs of the individual patient.[109] If there is major organ involvement in patients with pSS, azathioprine may be a better choice than leflunomide or sulfasalazine for the treatment of all complications including inflammatory musculoskeletal pain.[109]​

Vasculitis

The most common extra-glandular manifestation in patients with vasculitis is skin rash, usually palpable purpura. Most patients have only a single lifetime episode of skin vasculitis. Treatment is a short course of corticosteroids.[110] Intravenous immunoglobulin treatment in more severe/resistant cases may be effective, but therapeutic experience in vasculitis is limited.[110]

Rituximab may be considered for patients with pSS with vasculitis or cryoglobulinaemia associated with vasculitis manifestations if they have experienced unacceptable adverse effects or toxicity with corticosteroids or other agents, or they are not able to taper and discontinue corticosteroid treatment.[109]​

Renal tubular acidosis

A few patients may have severe manifestations of proximal renal tubular acidosis (RTA), even presenting with profound hypokalaemia. The usual therapy of type 2 RTA of any aetiology should be instituted. This includes potassium repletion and alkali by mouth. See Renal tubular acidosis.

Neuropathy

A substantial minority of patients have a peripheral sensory neuropathy. Occasionally the neuropathy is severe such that gait is impaired. There are several case reports and small case series in which intravenous immunoglobulin has been effective.[111][112][113]

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