Presentation

A 57-year-old woman presented with a 2-month history of bloody sputum, dyspnoea on exertion, and cough. The patient had a history of inhaling silica-based cleanser powder from the age of 16 to 36 years that started as a personal remedy for nausea and anorexia in early pregnancy, but later became a habit. She and her family had no history of tuberculosis. Raynaud's phenomenon was first noted 5 years ago, and shortness of breath appeared 2 years ago.

On physical examination, fine crackles of the left lower lobe, sclerodactyly of the distal fingers, periungual erythema, giant capillaries and bleeding at the nail fold (Fig. 1A, B), and telangiectasias on the extremities were observed; however, digital ulcers were absent. Laboratory examination revealed a positive centromere antibody of 175 U/mL. Computed tomography revealed multiple egg-shell calcified lymph nodes in the mediastinum and hilar region, as well as reticulonodular shadows in the left lung (Fig. 1C, D). The patient’s history of silica exposure and clinical findings were consistent with silicosis and systemic sclerosis (SSc), leading to the diagnosis of Erasmus syndrome. Her shortness of breath gradually deteriorated and echocardiography revealed elevated tricuspid regurgitant jet velocity in the next two years. She was subsequently diagnosed with comorbid pulmonary artery hypertension by right heart catheterisation.

Fig. 1
figure 1

(A) Sclerodactyly of the distal fingers, periungual erythema, periungual epithelial extensions, nail fold bleeding (arrows), and (B) nail fold capillaroscopy showing giant capillaries (arrowhead) and bleeding (arrow). Computed tomography showing multiple egg-shell calcified lymph nodes (C, D, arrows) in the mediastinum and hilar region, and reticulonodular shadows in the left lung (D, arrowheads)

Full size image

Discussion

Erasmus syndrome is a rare disease characterized by the development of SSc in a background of silica exposure or silicosis, which was first reported by Dr. Erasmus in 1957 [1]. Silicosis is a chronic and irreversible occupational lung disease, predominantly observed in miners and stone cutters [2]. Chronic silica exposure is a risk factor for the development of both silicosis [3] and SSc [4]. Pulmonary tuberculosis is an important differential diagnosis because of the similarities in respiratory symptoms and imaging findings [4, 5]. This case emphasizes the need to recognize that even people without occupational exposure to silica may develop Erasmus syndrome.