Unraveling complexity: A rare case of pulmonary sarcoidosis coinciding with systemic scleroderma

We present a rare case of a 66-year-old female with systemic sclerosis and Raynaud's phenomenon, diagnosed at age 25, who was found to have multinodular pulmonary sarcoidosis. The coexistence of sarcoidosis and scleroderma, are relatively common, well-documented in medical literatures however, the relationship between the two is complex and multifactorial. She was referred to pulmonology for evaluation of a lung mass and nodule noted on chest x-ray. Chest CT revealed widespread bilateral pulmonary nodularity, more prominent on the right, with multifocal mass-like consolidation. Differential diagnoses included malignancy, infection, or inflammatory disease. A transbronchial lung biopsy of the right upper and middle lobes showed focal granulomatous inflammation with negative AFB staining, and lymph node biopsies were negative for malignancy. These findings favored a diagnosis of sarcoidosis over systemic sclerosis, which typically does not present with granulomatous inflammation. A PET CT was performed to further differentiate between inflammation and malignancy, revealing extensive perilymphatic nodularity and FDG-avid mass-like opacities, especially in the right upper and middle lobes, consistent with the sarcoid Galaxy sign. She was initiated on prednisone 5 mg and methotrexate 25 mg daily. Given the rarity of this presentation, recognizing the co-occurrence of these two autoimmune conditions was critical in reaching the correct diagnosis and initiating appropriate therapy.