Sjögren's syndrome: a critical review of clinical management

Sjögren's syndrome (SS) is greatly under recognized in clinical practice, primarily for 2 reasons: its presentations are variable and often nonspecific and there are still no clear, uniform diagnostic criteria for this clinical entity. The prevalence, natural history, pathogenesis, and clinical taxonomy of SS are still not well understood. Potential criteria include both subjective symptoms and objective criteria such as measurements of salivary and tear flow, minor salivary gland biopsy, and an increasing variety of serological markers. Physicians often fail to appreciate the profound impact of SS on quality of life. Therefore, screening for SS should include questions exploring symptoms in terms of their effect on the patient's daily life. At present, there is no curative treatment for SS. For symptom relief, local treatments (such as artificial tears or oral topical sprays) are limited in their effects, whereas systemic treatment offers the advantage of addressing a wider range of symptoms. Controlled studies show that oral pilocarpine significantly improves sicca symptoms in the eyes, mouth, and other sites. Clinical experience to date suggests it is safe and well tolerated, with no serious adverse effects, tachyphylaxis, or drug to drug interactions of concern. The most frequent adverse effects are sweating, urinary frequency, diarrhea, and other parasympathomimetic effects, but these do not lead to substantial drug withdrawal rates. Patients should be forewarned that subjective improvement may lag behind improvement in objective measures. Because management often spans several specialties, coordination among them is essential. Dental, gynecological, and ophthalmological perspectives on diagnosis and management are discussed; the primary practitioner has the opportunity to play both a coordinating role and a direct role in early diagnosis and treatment.