Sjogren’s syndrome with multi-organ extraglandular manifestations - a case report

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Background: Primary Sjogren’s syndrome is an autoimmune disorder characterized by diminished lacrimal and salivary gland functions. Other than the exocrine gland involvement, it is also known to affect other visceral organs, resulting in extraglandular manifestations. Obvious cardiac involvement is rare, with pericardial effusions being the commonest feature. Sjogren’s syndrome is also known to cause renal involvement, with tubulointerstitial nephritis being the most typical. Case Presentation: We report of a 37-year-old female with primary Sjogren’s syndrome who developed bilateral parotid swelling, generalized edema, palpable purpura, arthralgias, and dyspnea. Skin biopsy of the purpuric lesions demonstrated leukocytoclastic vasculitis. Echocardiography revealed a low left ventricular ejection fraction (~45%) and a small pericardial effusion (10 mm). These findings together with an abnormally raised NT-proBNP of 2,424 pg/ml were highly suspicious for an autoimmune myocarditis. Proteinuria (1.8 g/24 hours) was present and renal biopsy confirmed membranoproliferative glomerulonephritis. Cryoglobulins were positive. Upon commencement of the treatment, with intravenous bumetanide, pulsed methylprednisolone, and enalapril, the patient experienced rapid symptom resolution. Conclusion: The extraglandular manifestations of primary Sjogren’s syndrome are many and may affect more than one organ at the same time. Although rare, autoimmune myocarditis is an important differential in Sjogren’s syndrome patients who present with dyspnea.