ERASMUS SYNDROME IN A GRANITE WORKER

TOPIC: Diffuse Lung Disease TYPE: Medical Student/Resident Case Reports INTRODUCTION: Erasmus syndrome is a rare entity in which systemic sclerosis develops following exposure to silica with or without silicosis. CASE PRESENTATION: A 51 year-old-male patient, non-smoker, with occupational exposure to silica dust for nearly 20 years, referred to the Rheumatology Department for moderate skin thickening over the face and extremities. He also reported to have Raynaud's phenomenon for 3 years. At this time, he had no dyspnea or dysphagia. The diagnosis of limited systemic sclerosis was determined according to his clinical history, the positivity of anti-Scl 70 and anti-P antibodies and changes in nailfold capillaroscopy. Three months later, he was admitted to the Pulmonology Department for dyspnea with moderate efforts and changes in chest CT – multiple small centrilobular confluent pulmonary nodules associated with hilar and mediastinal adenopathies with coarse calcifications. The bronchofibroscopy with bronchoalveolar lavage excluded infectious diseases or cancer. Pulmonary function tests detected an obstrutive pattern and reduced diffusing capacity on the lung for carbon monoxide. In the end, the occupational history and the findings from the diagnostic procedures established the diagnosis of silicosis. He started inhaled therapy, prednisone 10 mg daily and methotrexate 25 mg weekly with progressive recovery. DISCUSSION: Silica can activate monocytes to release cytokines IL-1, 6 and TNF alpha, which are fibroblast proliferative factors, stimulating collagen production and leading to cutaneous sclerosis, vascular occlusion and pulmonary fibrosis. CONCLUSIONS: Finally, this clinical case evidences that occupational exposure is related to an increase risk of severe diseases and primary prevention can be performed by using individual protection devices for professionals exposed. REFERENCE #1: Jalutharia J, Dixit R. Erasmus Syndrome: Co-existence of silicosis and progressive systemic sclerosis in a 55 year old male. J Assoc Chest Physicians 2020; 8:19-22 REFERENCE #2: Z.D. McCormic, S.S. Khuder, B.K. Aryal, A.L. Ames, S.A. Khuder. Occupation silica exposure as a risk factor for scleroderma: a meta analysis. In Arch Occup Environ Health., 83 (7) (2010), pp763-769 DISCLOSURES: No relevant relationships by Mariana Argel, source=Web Response No relevant relationships by Ana Campos, source=Web Response No relevant relationships by Mariana Conceicao, source=Web Response No relevant relationships by Ana Rita Ferro, source=Web Response No relevant relationships by Sónia Isabel Guerra, source=Web Response No relevant relationships by António Torres, source=Web Response