Clinical Pearls & Morning Reports
Published March 7, 2018
Primary Sjögren’s syndrome is a common systemic autoimmune disease, with a female-to-male predominance of 9:1 and peak incidence at approximately 50 years of age. The hallmark of the disease is exocrinopathy, which often results in dryness of the mouth and eyes, fatigue, and joint pain. These three symptoms are present in more than 80% of the patients with this disease. Read the latest NEJM Clinical Practice article here.
A: Systemic manifestations occur in approximately 30 to 40% of the patients with primary Sjögren’s syndrome. Lymphocytic infiltration of the epithelia of organs beyond the exocrine glands can cause interstitial nephritis, autoimmune primary biliary cholangitis, and obstructive bronchiolitis. Immune complex deposition as a result of the ongoing B-cell hyperreactivity can result in extraepithelial manifestations, such as palpable purpura, cryoglobulinemia-associated glomerulonephritis, interstitial pneumonitis, and peripheral neuropathy.
A: The risk of B-cell lymphoma is 15 to 20 times as high among patients with primary Sjögren’s syndrome as in the general population (lifetime risk, 5 to 10%), a finding that has been attributed to the chronic B-cell activation in this condition. These lymphomas are mostly B-cell non-Hodgkin’s lymphomas, with a predominance of the low-grade, marginal-zone histologic type. Lymphomas often develop in organs in which primary Sjögren’s syndrome is active, such as the salivary glands, and thus are primarily mucosa-associated lymphoid tissue (MALT) lymphomas.
A: Anti-SSA antibodies (often associated with antibodies against Sjögren’s syndrome–related antigen B [anti-SSB antibodies]) are present in two thirds of patients and should be assessed when primary Sjögren’s syndrome is suspected. Rheumatoid factor is present in approximately half of the patients, whereas antibodies against double-stranded DNA (important in the diagnosis of systemic lupus erythematosus) are typically absent. Biopsy of minor salivary glands is typically recommended for establishing a diagnosis of primary Sjögren’s syndrome in the absence of anti-SSA antibodies. Measures of oral and ocular dryness may also be useful. Ultrasonography of the major salivary glands may reveal multiple hypochoic or anechoic areas in the four main salivary glands (parotid and submandibular glands) and may be helpful in diagnosis or longitudinal assessment, although such evaluation is not formally included among the classification criteria.
A: A systematic review of randomized clinical trials has shown the benefit of muscarinic agonists (pilocarpine hydrochloride and cevimeline hydrochloride) for the treatment of oral dryness and, to a lesser extent, ocular dryness in patients with primary Sjögren’s syndrome. The use of topical cyclosporine eyedrops (0.05% or 0.1% concentration) has been shown to result in better tear production than placebo and in improvement in symptom scores among patients with moderate or severe ocular dryness and inflammation, although such findings have been mixed. Randomized trials assessing the efficacy of analgesic drugs in primary Sjögren’s syndrome are lacking. On the basis of clinical experience, simple analgesics (e.g., acetaminophen) may be used as first-line therapy for pain; nonsteroidal antiinflammatory drugs may be appropriate for joint pain. Neuropathic pain in patients with primary Sjögren’s syndrome is typically treated with gabapentin, pregabalin, or duloxetine, which are associated with less dryness of the mouth and eyes than small doses of amitriptyline. No drug has been shown to be effective for the fatigue that is so common among patients with primary Sjögren’s syndrome. Severe organ manifestations are treated in accordance with guidelines for systemic lupus erythematosus or other connective-tissue diseases.