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Is Interstitial Lung Disease Hereditary?

Interstitial lung disease is a group of lung disorders that affect the tissues and spaces around the alveoli (air sacs) of the lungs, called the interstitium. It describes more than 200 different lung diseases/disorders that damage the lung tissue. It is relatively more common than previously thought. A person is thought to be genetically predisposed to interstitial lung disease. It shows a pattern of hereditary/inheritance of an autosomal recessive gene that is associated with inborn errors of metabolism. A younger age of onset of disease also reflects genetic anticipation, which is generally seen in autosomal dominant kindreds with inherited telomerase mutations.

The most common disorders affecting the lungs are pulmonary fibrosis, occupational diseases, connective tissue interstitial disease and sarcoidosis (it is the growth of tiny collections of inflammatory cells called as granulomas). They affect the normal absorption of oxygen in the lungs.

Interstitial lung disease is a chronic, non-malignant and non-infectious disease in nature. The symptoms vary from person to person depending on the advancement of the disease. It usually affects people older than 40 years of age. The disease is progressive and usually leads to pulmonary hypertension and right heart failure. It may also cause large airway obstruction, mediastinal, bronchial or intraparenchymal neurofibromas, intercostals tumors and abnormal curvature of the spine.

The known causes of interstitial lung disease are radiation exposure, connective tissue disorders, sarcoidosis, various drugs (chemotherapeutic drugs, antibiotics such as ciprofloxacin and certain psychiatric medications) and genetic conditions. Exposure to toxic agents such as asbestos, silica, wood dusts and antigens can lead to interstitial lung disease. Certain viral infections like cytomegalovirus, which occurs mostly in HIV patients, bacterial infections like pneumonia, fungal infections and parasitic infections can cause pulmonary infections leading to interstitial lung disease. Medical conditions such as connective tissue disorders and hematological diseases like systemic lupus erythematosus, rheumatoid arthritis, polymyositis and Sjogren’s syndrome, all are causative factors for interstitial lung disease.

Interstitial pneumonitis is the most common idiopathic interstitial lung disease that accounts for more than half the cases of ILD. It mostly affects men as compared to women especially in the age group of 40-70 years. People who smoke tobacco regularly are at a higher risk of developing interstitial lung disease than people who do not.

The symptoms are dyspnea or shortness of breath, dry cough without sputum. In case of prolonged disease swelling of the legs and heart failure might also occur. Other symptoms are wheezing, weight loss, cyanosis (bluish discoloration of the skin and mucus membrane due to lack of oxygen in blood) and clubbing of the fingers.

Diagnosis And Treatment Of Interstitial Lung Disease

Interstitial lung disease is diagnosed with the help of pulmonary function tests, high resolution computerized tomography, exercise tests, transbronchial biopsy, bronchioalveolar lavage (fluid sample from the air sacs is taken), chest X ray, video assisted thoracoscopic surgery (to confirm the diagnosis) and blood gases test where the amount of oxygen present in the blood is determined.

Treatment of interstitial lung disease is easier when it is in the early stage. Patients who smoke tobacco should be encouraged to quit if they want better results.

Antifibrotics reduce the development of scar tissue and help slow the progression of the disease. Colchicines and D-penicillamine have shown positive results in most cases. Corticosteroids reduce inflammation in the lungs, but in the long run they cause glaucoma, bone loss, high blood sugar, and poor wound healing and increased susceptibility to infection.

A lung transplant is necessary for patients who have severe interstitial lung disease and who do not respond to other treatment options. In order to receive a transplant a person should be healthy and be willing to follow the medical program. Oxygen therapy makes breathing and exercise easier for interstitial lung disease patients.

Pulmonary rehabilitation is done for patients with chronic lung disease, providing medical care and support. It mainly focuses on exercises including breathing techniques, as well as education, emotional support and nutritional counseling.

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Is Interstitial Lung Disease Hereditary?