What autoimmune diseases cause interstitial lung disease
What autoimmune diseases cause interstitial lung disease Autoimmune diseases are a group of disorders in which the immune system mistakenly attacks the body’s own tissues. One of the serious complications associated with certain autoimmune conditions is interstitial lung disease (ILD), a broad term describing a range of disorders that cause inflammation and scarring of the lung interstitium—the tissue and space around the air sacs of the lungs. The relationship between autoimmune diseases and ILD is complex, as many autoimmune conditions can lead to pulmonary involvement, significantly impacting patient outcomes.
Several autoimmune diseases are well-known for their potential to cause interstitial lung disease. Among these, rheumatoid arthritis (RA) is one of the most common. RA primarily affects joints but can also involve extra-articular tissues, including the lungs. Pulmonary manifestations in RA patients can range from mild inflammation to extensive fibrosis. The mechanism involves chronic inflammation that damages the lung interstitium, leading to symptoms such as cough, shortness of breath, and decreased lung function.
Systemic sclerosis, also known as scleroderma, is another autoimmune disease strongly associated with ILD. It is characterized by fibrosis of the skin and internal organs, including the lungs. Interstitial lung disease in systemic sclerosis can be aggressive and is a leading cause of mortality in these patients. The fibrotic process is driven by abnormal immune responses and excessive collagen deposition, which thickens and stiffens the lung tissue, impairing gas exchange.
Another significant autoimmune condition linked to ILD is polymyositis and dermatomyositis, collectively known as idiopathic inflammatory myopathies. These diseases primarily affect muscles but can also involve the lungs, leading to an interstitial pattern of lung injury. Patients often present with cough, dyspnea, and muscle weakness, and early detection is crucial for management.
Systemic lupus erythematosus (SLE) also has pulmonary manifestations, although ILD is less common compared to other lung issues like pleuritis or pulmonary hypertension. When ILD occurs in SLE, it can cause significant respiratory symptoms and may involve various patterns, i
ncluding nonspecific interstitial pneumonia (NSIP) or usual interstitial pneumonia (UIP). The immune complexes and autoantibodies in SLE contribute to inflammation and fibrosis in the lung tissue.
Other autoimmune conditions such as Sjögren’s syndrome, mixed connective tissue disease (MCTD), and vasculitides like granulomatosis with polyangiitis can also involve the lungs with interstitial patterns. In these diseases, autoantibodies and immune-mediated inflammation play roles in damaging the interstitial tissue, leading to fibrosis over time.
In clinical practice, identifying the underlying autoimmune disease is vital for managing ILD effectively. Treatment often involves immunosuppressive medications like corticosteroids, cyclophosphamide, or newer biologic agents aimed at controlling the autoimmune process and preventing further lung damage. Early diagnosis and intervention are crucial, as progressive fibrosis can lead to significant respiratory impairment and reduced quality of life.
In summary, several autoimmune diseases, including rheumatoid arthritis, systemic sclerosis, polymyositis, dermatomyositis, and SLE, are known to cause interstitial lung disease. Understanding the link between autoimmunity and lung pathology helps in early detection, tailored treatment, and improving prognosis for affected individuals.
