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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
From Silicosis to Systemic Sclerosis: Erasmus Syndrome in a Young Galamseyer-Case Report
Solomon Gyabaah1, Seth Kyei-Fram1, Kojo Awotwi Hutton Mensah1,2
1Department of Medicine, Komfo Anokye Teaching Hospital, Ashanti Region, Kumasi, Ghana, kathhsp.org.
Abstract:
Erasmus syndrome is an uncommon condition marked by the onset of systemic sclerosis (SSc) due to extended exposure to silica. In this case, we highlight the significant health risks associated with silica exposure in artisanal mining (galamsey), leading to the development of Erasmus syndrome. We present the case of a 33-year-old male artisanal miner with a 14-year work history who presented with a 2-year history of recurrent cough, difficulty breathing, and fever. He later developed hypopigmentation and skin thickening on his face, neck, torso, and knees, along with a productive cough and low-grade fever. He visited multiple healthcare facilities on account of the above complaints. At one of such visits, he was prescribed antituberculosis (TB) medication even though the sputum for GeneXpert was negative. On examination, he was in respiratory distress with an oxygen saturation of 78% on room air and required oxygen via a nonrebreather mask at 10 L/min, which raised the oxygen saturation to 94%, and had bronchial breath sounds in the right lung zones. Vocal resonance was increased bilaterally in the middle lung zones. He had salt-and-pepper dermopathy with tight and adherent skin on his face, chest, back, and knees. He additionally had microstomia, rat bite sign of the fingers, and Raynaud's phenomenon. Antinuclear antibody by indirect immunofluorescence on hep-2 cells was positive (1:320), and Antitopoisomerase 1 antibodies were also positive. Chest CT findings were consistent with complicated silicosis. The patient was diagnosed with Erasmus syndrome and started on immunosuppressive therapy. We highlight the significant health risks associated with silica exposure in artisanal mining (galamsey), leading to the development of Erasmus syndrome. Preventing silica exposure remains crucial, necessitating stringent workplace safety measures and occupational health practices to protect individuals in high-risk professions.
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