Case Report

Volume: 4 | Issue: 12 | Published: Dec 24, 2020 | Pages: 414 - 418 | DOI: 10.24911/ejmcr/173-1565175435

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

Authors: Clarissa-Marie Zehlicke , Christian Vassallo , Wei Li Chan , Ritienne Debono , Bernard Coleiro

Article Info

Authors

Clarissa-Marie Zehlicke

Department of Medicine, Mater Dei Hospital, Msida, Malta

Christian Vassallo

Department of Rheumatology, Mater Dei Hospital, Msida, Malta

Wei Li Chan

Department of Medicine, Mater Dei Hospital, Msida, Malta

Ritienne Debono

Department of Nephrology, Mater Dei Hospital, Msida, Malta

Bernard Coleiro

Department of Rheumatology, Mater Dei Hospital, Msida, Malta

Publication History

Received: January 08, 2020

Revised: October 03, 2020

Accepted: October 21, 2020

Published: December 24, 2020

Abstract

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.

Keywords: Sjogren’s syndrome, cryoglobulinemia, vasculitis, myocarditis, glomerulonephritis, case report, green

Open access Creative Commons License

Pubmed Style

Clarissa-Marie Zehlicke, Christian Vassallo, Wei Li Chan, Ritienne Debono, Bernard Coleiro. Sjogren’s syndrome with multi-organ extraglandular manifestations - a case report. EJMCR. 2020; 24 (December 2020): 414-418. doi:10.24911/ejmcr/173-1565175435

Web Style

Clarissa-Marie Zehlicke, Christian Vassallo, Wei Li Chan, Ritienne Debono, Bernard Coleiro. Sjogren’s syndrome with multi-organ extraglandular manifestations - a case report. https://ejmcr.com/articles/1361 [Access: December 25, 2024]. doi:10.24911/ejmcr/173-1565175435

AMA (American Medical Association) Style

Clarissa-Marie Zehlicke, Christian Vassallo, Wei Li Chan, Ritienne Debono, Bernard Coleiro. Sjogren’s syndrome with multi-organ extraglandular manifestations - a case report. EJMCR. 2020; 24 (December 2020): 414-418. doi:10.24911/ejmcr/173-1565175435

Vancouver/ICMJE Style

Clarissa-Marie Zehlicke, Christian Vassallo, Wei Li Chan, Ritienne Debono, Bernard Coleiro. Sjogren’s syndrome with multi-organ extraglandular manifestations - a case report. EJMCR. (2020), [cited December 25, 2024]; 24 (December 2020): 414-418. doi:10.24911/ejmcr/173-1565175435

Harvard Style

Clarissa-Marie Zehlicke, Christian Vassallo, Wei Li Chan, Ritienne Debono, Bernard Coleiro (2020) Sjogren’s syndrome with multi-organ extraglandular manifestations - a case report. EJMCR, 24 (December 2020): 414-418. doi:10.24911/ejmcr/173-1565175435

Chicago Style

Clarissa-Marie Zehlicke, Christian Vassallo, Wei Li Chan, Ritienne Debono, Bernard Coleiro. "Sjogren’s syndrome with multi-organ extraglandular manifestations - a case report." 24 (2020), 414-418. doi:10.24911/ejmcr/173-1565175435

MLA (The Modern Language Association) Style

Clarissa-Marie Zehlicke, Christian Vassallo, Wei Li Chan, Ritienne Debono, Bernard Coleiro. "Sjogren’s syndrome with multi-organ extraglandular manifestations - a case report." 24.December 2020 (2020), 414-418. Print. doi:10.24911/ejmcr/173-1565175435

APA (American Psychological Association) Style

Clarissa-Marie Zehlicke, Christian Vassallo, Wei Li Chan, Ritienne Debono, Bernard Coleiro (2020) Sjogren’s syndrome with multi-organ extraglandular manifestations - a case report. , 24 (December 2020), 414-418. doi:10.24911/ejmcr/173-1565175435