Interstitial lung disease or ILD can be described as inflammation, fibrosis, or scarring of the lung’s interstitial, resulting in dyspnea. ILD represents a group of heterogeneous parenchymal lung disorders with complex pathophysiology, differentiated by the clinical and radiological patterns. ILD is one of the most serious pulmonary complications associated with connective tissue diseases (CTDs), resulting in significant morbidity and mortality. Nonspecific interstitial pneumonia is the most common morphological and pathological pattern of ILD seen in CTDs. There are limitations in the therapeutic options resulting in significant morbidity. Certain biologic therapies are being evaluated for the various forms of ILD. The ILD, in this case, is associated with systemic lupus erythematosus (SLE) and scleroderma overlap that was effectively treated with belimumab, a recombinant monoclonal antibody against the B-cell activating factor (B-lymphocyte stimulator).
Dermatomyositis (DM) and polymyositis (PM) are idiopathic inflammatory myopathies. Interstitial lung disease (ILD) develops in most patients with DM and PM directly related to morbidity and mortality. Diagnosis requires a myositis panel and high-resolution computed tomography (HRCT). Prognosis depends on specific myositis-specific antibodies and the pattern of the interstitial lung changes. Anti-Mi-2 antibody-specific dermatomyositis has a lower prevalence of interstitial lung disease and has a favorable prognosis, responding well to steroids. Our patient is a 72-year old male who presented with recurrent episodes of pneumonitis, and ILD was found to have anti-Mi-2 beta-specific dermatomyositis and SLE overlap disease. He was responding well to high-dose steroids but was rebounding to similar symptoms whenever steroid weaning was attempted. He was started on azathioprine, but unfortunately, his disease rapidly progressed, and he died within a few months. This manuscript enhances the temporal relationship between dermatomyositis and ILD.
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