Recent Developments in the Diagnosis and Management of Sjögren's Syndrome

Chung-Tei Chou

Disclosures

Int J Clin Rheumatol. 2011;6(4):445-452. 

In This Article

Abstract and Introduction

Abstract

Sjögren's syndrome (SS) is a common immune disease that mainly affects the exocrine glands, which clinically present xerostomia, keratoconjuctivitis sicca and serological autoantibodies including rheumatoid factor, antinuclear antibody, anti-SS-A and anti-SS-B. The prevalence of SS is high but underestimated in the general population. SS may involve many other organs and tissues including lung (interstitial lung disease), kidney (renal tubular acidosis and hypokalemia), nerve (peripheral neuropathy), vessel (vasculitis, Raynauld's phenomenon), bladder (interstitial cystitis), lymph node (lymphaenopathy), liver (autoimmune hepatitis), pancreas (pancreatitis) and GI (reflux esophagitis, peptic ulcer). In 2010, a new disease activity, the European League Against Rheumatism (EULAR) Sjögren Syndrome Disease Activity Index (ESSDAI) was proposed. Diagnosis of SS is based upon the 2002 classification criteria of SS, which originated from the 1996 European classification criteria. Methods for diagnosis of SS include salivary flow assessment, salivary gland scintigraphy, Schimer's test, Rose Bengal test and minor salivary gland biopsy. Treatment of dry mouth and dry eye requires muscarinic agonists, for which two drugs are now available; pilocarpine and cevimeline. Hydroxychloroquine is not useful for sicca syndrome, however, it may be effective to relieve arthralgia. Active immunotherapy is considered when the patient has pulmonary, neurologic and renal involvement. Corticosteroid, cyclophosphamide, azathioprine, mycophenolate mofetal (cellcept), mizoribine and cyclosporine are the immunosuppressive drugs that have been used for SS with systemic manifestations. TNF-α blocker has been used in SS but, in general, it demonstrated no benefit to relieve oral or eye dryness. More recently, rituximab, a B cell directed therapy, was reported to have efficacy for sicca symptoms or pulmonary, neurologic involvement. In this article, we demonstrate current diagnosis and therapy for SS.

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