Multiple-autoimmune syndrome causing interstitial lung disease in the presence of pulmonary hypertension and Erasmus syndrome

Abstract

Connective tissue diseases constitute a group of inflammatory disorders that can concurrently affect multiple organs. Pulmonary manifestations of connective tissue diseases include interstitial lung disease, pulmonary hypertension, pleural diseases, and airway compromise. Both interstitial lung disease and pulmonary arterial hypertension have high morbidity and mortality in these patients. Interstitial lung disease affects 40 % - 50 % of patients with connective tissue diseases, predominantly systemic sclerosis, rheumatoid arthritis, and inflammatory myopathies. Parenchymal compromise is heterogeneous and depends on the underlying disease. Conversely, pulmonary arterial hypertension is found mostly in systemic sclerosis, systemic lupus erythematosus and mixed connective tissue disease. Delays and the diagnosis of connective tissue disorder associated interstitial lung disease are frequent as evidence is limited and as they often present concomitantly or overlap with other pulmonary diseases. Timely diagnosis and treatment are fundamental to decrease mortality. Treatment varies by the type of connective tissue disease, nevertheless, evidence of these complications in the context of multiple-autoimmune syndrome is still limited.
 We present a complex case of a 47-year-old male with a multiple-autoimmune syndrome (systemic lupus erythematosus, rheumatoid arthritis, systemic sclerosis, Sjögren syndrome and mixed connective tissue disease) complicated by interstitial lung disease and pulmonary hypertension, and occurring concurrently with silicosis (Erasmus syndrome), and pulmonary embolism. This case shows the diverse manifestations of connective tissue of the lungs, emphasizes the intricate nature of diagnosing and managing connective tissue disorder-related pulmonary complications in the context of multiple autoimmune syndromes, and the importance of a multidisciplinary team for the diagnostic and therapeutic approach of the patient.