The treatment regimen depends on the affected target organs and disease severity. Sun exposure should be minimized and include use of a sunscreen. Patients are treated to promote clinical well-being, using serologic markers of disease activity as guidelines, including serum complement levels. Nonsteroidal anti-inflammatory agents, used to treat arthralgia and arthritis, are used with caution because patients with lupus are more susceptible to hepatotoxicity. Hydroxychloroquine is often used to treat mild manifestations including skin lesions, fatigue, arthritis, and arthralgia. Hydroxychloroquine may also reduce the risk of thromboembolic disease and lowers lipid levels.

Patients with thrombosis and antiphospholipid antibodies or a lupus anticoagulant should receive anticoagulant medication at least until lupus is in remission. The length of therapy is controversial. Low molecular weight heparin is the anticoagulant of choice; warfarin can also be used.

Corticosteroids control symptoms and autoantibody production in lupus. Treatment with corticosteroids has improved kidney disease and the rate of survival. Corticosteroids can make the diagnosis and treatment of tuberculosis difficult; all patients should have PPD and control skin tests, when possible, before corticosteroids are initiated. The optimal dose and route of administration of corticosteroids are controversial. Patients with systemic disease are often started on 1–2 mg/kg/24 hr of oral prednisone in divided daily doses. When complement levels increase to within the normal range, the dose is carefully tapered to the lowest effective dose. One method uses alternate-day high-dose corticosteroids once disease is controlled to prevent the Continue reading »

Sjögren syndrome is a chronic, inflammatory, autoimmune disease characterized by progressive lymphocytic and plasma cell infiltration of the salivary and lacrimal glands.

EPIDEMIOLOGY.

Sjögren syndrome typically presents at 35–45 yr of age, with 90% of cases among women. It is uncommon in the pediatric age group. Sjögren syndrome can occur as an isolated disorder, referred to as primary Sjögren syndrome (sicca complex), or as a secondary form in association with other rheumatic disorders.

ETIOLOGY AND PATHOGENESIS.

The etiology of Sjögren syndrome is complex and includes genetic predisposition, and possibly an infectious trigger. Lymphocytes and plasma cells infiltrate salivary glands, forming distinct periductal and periacinar foci that become confluent and may replace epithelial structure. This autoimmune exocrinopathy results in xerophthalmia (dry eyes, or keratoconjunctivitis sicca) and xerostomia (dry mouth). Several genes regulating apoptosis influence the chronicity of lymphocytic infiltration.

CLINICAL MANIFESTATIONS.

International classification criteria for the diagnosis of Sjögren syndrome in adult patients have been developed, and diagnostic criteria in children have been proposed. Clinical manifestations are related to exocrine disease of the epithelial surfaces of the eyes, mouth, nose, larynx and trachea, vagina, and skin, leading to the common symptoms of photophobia, burning and itching eyes, blurred vision, painless unilateral or bilateral enlargement of the parotid glands, decreased sense of taste, dental caries, dysphagia, fissured tongue, and angular cheilitis. At the onset of the disease, recurrent parotid gland enlargement and parotitis is the most common manifestation in children, Continue reading »