Pulmonary hemorrhage as a first manifestation of Sjögren’s disease in a young male adult

Sjögren's syndrome (SS) is a chronic autoimmune disorder primarily affecting exocrine glands, characterized by dry mouth (xerostomia) and dry eyes (keratoconjunctivitis sicca). However, systemic manifestations, including pulmonary complications, are increasingly recognized. Diffuse alveolar hemorrhage (DAH) is a rare but life-threatening presentation of SS, typically associated with vasculitis or coagulation abnormalities. We report the case of a 19-year-old male presenting with dyspnea and hemoptysis, later diagnosed with primary Sjögren's syndrome (pSS). Initial clinical assessment revealed respiratory failure with low blood oxygen levels (hypoxemia), bilateral ground-glass opacities on chest computed tomography (CT), and iron-storing macrophages (hemosiderin-laden) in bronchoalveolar lavage fluid, consistent with DAH. Subsequent autoimmune serology confirmed positivity for anti-Ro/Sjögren's-syndrome-related antigen A autoantibodies (SSA) and anti-La/Sjögren's-syndrome-related antigen B autoantibodies (SSB), while other autoimmune markers, including anti-neutrophil cytoplasmic antibodies (ANCA), were negative. Labial salivary gland biopsy demonstrated focal lymphocytic sialadenitis, confirming pSS according to European League Against Rheumatism (EULAR) criteria. Treatment involved high-dose corticosteroids, leading to complete resolution of symptoms and significant improvement in imaging results. The patient remained stable at follow-up. DAH should be considered a potential presentation of pSS, even in the absence of classical symptoms. Increased awareness of this rare complication can facilitate early diagnosis and improve outcomes.