Sjogren’s syndrome with multi-organ extraglandular manifestations - a case report
Authors: Clarissa-Marie Zehlicke, Christian Vassallo, Wei Li Chan, Ritienne Debono, Bernard Coleiro
Background: Primary Sjogrens 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. Sjogrens 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 Sjogrens 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 Sjogrens syndrome are many and may affect more than one organ at the same time. Although rare, autoimmune myocarditis is an important differential in Sjogrens syndrome patients who present with dyspnea.
Keywords: Sjogren’s syndrome, cryoglobulinemia, vasculitis, myocarditis, glomerulonephritis, case report
Authors
Correspondence to:
Clarissa Marie Zehlicke, Core Medical Trainee, Mater Dei Hospital, Triq Dun Karm, Imsida, Malta clarissazehlicke@gmail.com
Publication history:
Received 08 Jan 2020
Revised 03 Oct 2020
Accepted 21 Oct 2020
Published online 24 Dec 2020
Published in print 29 Dec 2020
Zehlicke C, Vassallo C, Chan WL, Debono R, Coleiro B. Sjogren’s syndrome with multi-organ extraglandular manifestations - a case report. EJMCR. 2020; 4(12): 414-418. doi:
10.24911/ejmcr/173-1565175435
Zehlicke C, Vassallo C, Chan WL, Debono R, Coleiro B. Sjogren’s syndrome with multi-organ extraglandular manifestations - a case report. https://www.ejmcr.com/?mno=60516 [Access: April 26, 2024]. doi:
10.24911/ejmcr/173-1565175435
Zehlicke C, Vassallo C, Chan WL, Debono R, Coleiro B. Sjogren’s syndrome with multi-organ extraglandular manifestations - a case report. EJMCR. 2020; 4(12): 414-418. doi:
10.24911/ejmcr/173-1565175435
Zehlicke C, Vassallo C, Chan WL, Debono R, Coleiro B. Sjogren’s syndrome with multi-organ extraglandular manifestations - a case report. EJMCR. (2020), [cited April 26, 2024]; 4(12): 414-418. doi:
10.24911/ejmcr/173-1565175435
Zehlicke, C., Vassallo, . C., Chan, . W. L., Debono, . R. & Coleiro, . B. (2020) Sjogren’s syndrome with multi-organ extraglandular manifestations - a case report. EJMCR, 4 (12), 414-418. doi:
10.24911/ejmcr/173-1565175435
Zehlicke, Clarissa-Marie, Christian Vassallo, Wei Li Chan, Ritienne Debono, and Bernard Coleiro. 2020. Sjogren’s syndrome with multi-organ extraglandular manifestations - a case report. European Journal of Medical Case Reports, 4 (12), 414-418. doi:
10.24911/ejmcr/173-1565175435
Zehlicke, Clarissa-Marie, Christian Vassallo, Wei Li Chan, Ritienne Debono, and Bernard Coleiro. "Sjogren’s syndrome with multi-organ extraglandular manifestations - a case report." European Journal of Medical Case Reports 4 (2020), 414-418. doi:
10.24911/ejmcr/173-1565175435
Zehlicke, Clarissa-Marie, Christian Vassallo, Wei Li Chan, Ritienne Debono, and Bernard Coleiro. "Sjogren’s syndrome with multi-organ extraglandular manifestations - a case report." European Journal of Medical Case Reports 4.12 (2020), 414-418. Print. doi:
10.24911/ejmcr/173-1565175435
Zehlicke, C., Vassallo, . C., Chan, . W. L., Debono, . R. & Coleiro, . B. (2020) Sjogren’s syndrome with multi-organ extraglandular manifestations - a case report. European Journal of Medical Case Reports, 4 (12), 414-418. doi:
10.24911/ejmcr/173-1565175435