Pericardial Effusion and Fibrotic Nonspecific Interstitial Pneumonia as Initial Manifestations of Primary Sjögren’s Syndrome: A Case Report

Mehdi Maaroufi; Hanaa Harraz; Khadija Chaanoun; Hanane Benjelloun; Nahid Zaghba

doi:10.36948/ijfmr.2026.v08i04.84336

Pericardial Effusion and Fibrotic Nonspecific Interstitial Pneumonia as Initial Manifestations of Primary Sjögren’s Syndrome: A Case Report

Author(s)	Dr. Mehdi Maaroufi, Prof. Dr. Hanaa Harraz, Prof. Dr. Khadija Chaanoun, Prof. Dr. Hanane Benjelloun, Prof. Dr. Nahid Zaghba
Country	Morocco
Abstract	Primary Sjogren's syndrome is a systemic autoimmune disease mainly characterized by lymphocytic involvement of exocrine glands, but extraglandular manifestations may occasionally dominate the clinical presentation. Pulmonary involvement is one of the most clinically relevant systemic manifestations, whereas pericardial involvement remains uncommon. We report the case of a 70-year-old woman with no toxic exposure who presented with a five-month history of progressive exertional dyspnea and a two-year history of subjective ocular dryness. Physical examination revealed bilateral basal crackles and a pericardial friction rub without signs of cardiac tamponade. Chest computed tomography showed a fibrotic nonspecific interstitial pneumonia pattern associated with bronchiectasis and a moderate pericardial effusion. Echocardiography confirmed a moderate circumferential pericardial effusion with preserved biventricular function and no hemodynamic compromise. Electrocardiography was normal and cardiac biomarkers were within normal limits. Immunological testing revealed positive antinuclear antibodies and anti-SSA antibodies. Ophthalmological assessment confirmed severe ocular dryness, while minor salivary gland biopsy was non-diagnostic. After exclusion of infectious, granulomatous, malignant and other systemic causes, primary Sjogren's syndrome with pulmonary involvement and probable pericarditis was retained. The patient improved under oral corticosteroids followed by mycophenolate mofetil as a steroid-sparing agent, with clinical, functional, radiological and echocardiographic improvement at one-year follow-up. This case highlights the importance of considering primary Sjogren's syndrome in patients with unexplained interstitial lung disease associated with pericardial effusion, even when sicca symptoms are limited.
Keywords	Primary Sjögren’s Syndrome, Interstitial Lung Disease, Nonspecific Interstitial Pneumonia, Pericardial Effusion, Case Report
Field	Medical / Pharmacy
Published In	Volume 8, Issue 4, July-August 2026
Published On	2026-07-24
DOI	https://doi.org/10.36948/ijfmr.2026.v08i04.84336

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Pericardial Effusion and Fibrotic Nonspecific Interstitial Pneumonia as Initial Manifestations of Primary Sjögren’s Syndrome: A Case Report

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