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Triple Overlap Between Silicosis, Dermatomyositis, and Systemic Sclerosis: Opening Up a Pandora's Box.

Siddharth Jain,J. Samanta,S. Vyas,S. Mahajan,V. Suri,U. Kumar

Published 2020 in Journal of clinical rheumatology

ABSTRACT

A 28-year-old stonemason presented with complaints of Raynaud phenomenon for 3 years, skin tightening for 2 years, and progressive dyspnea (modified Medical Research Council grade 2] for 1.5 years. He was diagnosed as having scleroderma with interstitial lung disease (ILD) and pulmonary hypertension at a regional facility and started on low-dose glucocorticoids, mycophenolate mofetil, and tadalafil. The patient was referred to us in view of worsening dyspnea (modified Medical Research Council grade 4) and new-onset muscle weakness. On examination, his oxygen saturation was 92% on room air. Modified Rodnan Skin Score was 34/51. Ulcerated Gottron papules were noted over the knuckles. Cardiopulmonary evaluation revealed bibasilar Velcro-like crackles and loud P2. Muscle power was Medical Research Council grade 4/5 in proximal upper and lower limbs bilaterally, with neck and truncal weakness. Investigations revealed elevated creatinine phosphokinase (2056 IU/L) and high-titer antinuclear (>140 U) and anti–Scl-70 (121.7 U) antibodies. Electromyography showed a myopathic pattern with increased insertional activity. Vastus lateralis muscle biopsy revealed variation in fiber size, perifascicular atrophy, perifascicular and perivascular inflammation, and granular membrane attack complex deposits on endomysial blood vessels (Figs. A and B). A diagnosis of scleroderma/dermatomyositis overlap was considered. Pulmonary function testing showed marked reduction in forced vital capacity (43%). Echocardiography revealed mild pulmonary hypertension. Extensive fibrosis with bilateral reticulonodular opacities was seen on the chest radiograph. Computed tomography of the chest showed mediastinal lymphadenopathy with eggshell calcification and upper-lobe predominant pulmonary fibrosis with pleural thickening (Figs. C–E). In retrospect, the patient reported a significant workplace exposure to silica for the past 7 years. He was (re)-diagnosed as a case of triple overlap between silicosis, dermatomyositis, and scleroderma and started on oral glucocorticoids (1 mg/kg per day) and methotrexate. Thus, what was initially treated as connective tissue disease (CTD)– ILD was actually silicosis. On follow-up, he reported significant improvement in muscle power and skin tightening but persistent respiratory complaints.

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