Diagnostic problems in a patient with pulmonary fibrosis and soft tissue calcifications: Scleromyositis

Abstract

Overlap syndromes are a spectrum of disorders with mixed clinical features of different connective tissue diseases, which often make the diagnostic process difficult. The article describes the case of a 57-year-old woman with arthralgia, interstitial lung disease and soft tissue calcifications. Despite the absence of any typical skin lesions, predominant clinical features of pulmonary fibrosis and vascular changes in the nailfold capillaroscopy were strongly suggestive of scleroderma-like syndrome. During the course of the disease, six months later, laboratory tests revealed elevated creatine phosphokinase and electromyography confirmed features of primarymuscle damage. Since muscle involvement and soft tissue calcifications might be characteristic for scleromyositis, a cutaneous-muscular biopsy was performed to confirm the diagnosis.We speculate that early development of calcifications in this case could be accelerated by vitamin D deficiency and secondary hyperparathyroidism. Small doses of glucocorticosteroids, azathioprine and supplementation of vitamin D were sufficient to control clinical symptoms of the disease.