REVUE ALGERIENNE D’ALLERGOLOGIE
Volume 11, Numéro 1, Pages 20-24
2026-03-05

Pulmonary Fibrosis In Systemic Sclerosis And Granulomatosis With Polyangiitis: A Case Report



Authors : Lesmar Ahlem . Mokrane Zine Labidine . Alioua Amina . Chaouki Feryel .

Abstract

Introduction : Pulmonary fibrosis is a major cause of morbidity and mortality in systemic autoimmune diseases. Systemic sclerosis (SSc) is frequently associated with interstitial lung disease, whereas granulomatosis with polyangiitis (GPA) rarely presents with fibrotic lung involvement. The coexistence of these two conditions constitutes an exceptional overlap syndrome and poses diagnostic and therapeutic challenges. Methods : We report the case of a 64-year-old male presenting with progressive dyspnea and chronic cough evolving over three years, associated with nasal congestion, Raynaud’s phenomenon, and skin thickening. High-resolution computed tomography (HRCT) revealed a non-specific interstitial pneumonia (NSIP) pattern. Immunological investigations showed positivity for anti-Scl-70 and anti-proteinase 3 (PR3) antibodies. Results : A multidisciplinary assessment established the diagnosis of pulmonary fibrosis secondary to overlapping SSc and GPA. Treatment with mycophenolate mofetil resulted in partial clinical improvement and stabilization of respiratory symptoms. Conclusion : This case highlights the importance of systematic autoimmune evaluation in interstitial lung disease and illustrates the role of individualized immunosuppressive therapy in rare overlap syndromes involving both fibrotic and vasculitic mechanisms.

Keywords

Pulmonary Fibrosis; ; Systemic Sclerosis; ; Granulomatosis with Polyangiitis; ; Overlap Syndrome; ; Interstitial Lung Disease; ; ANCA-Associated Vasculitis.

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