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What is
Insterstitial Lung Disease?
Interstitial lung disease (ILD), also known as diffuse parenchymal lung disease (DPLD), refers to a group of lung diseases affecting the interstitium (the tissue and space around the air sacs of the lungs), that inflame or scar the lungs. It concerns alveolar epithelium, pulmonary capillary endothelium, basement membrane, perivascular and perilymphatic tissues.
Prolonged ILD may result in pulmonary fibrosis which make it hard to get enough oxygen. The term Idiopathic pulmonary fibrosis is used to describe interstitial lung disease for which no obvious cause can be identified , and is associated with typical radiographic (basal and pleural based fibrosis with honeycombing) and pathologic (temporally and spatially heterogeneous fibrosis, histopathologic honeycombing and fibroblastic foci) findings.
What is Interstitium?
The interstitium is a lace-like network of tissue that extends throughout both lungs. The interstitium provides support to the lungs’ microscopic air sacs (alveoli). Tiny blood vessels travel through the interstitium, allowing gas exchange between blood and the air in the lungs. Normally, the interstitium is so thin it can’t be seen on chest X-rays or CT scans.
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What causes ILD?
- Inhaled substances
- Inorganic
- Silicosis
- Asbestosis
- Berylliosis
- Organic:
- Hypersensitivity pneumonitis
- Drug induced
- Antibiotics
- Chemotherapeutic drugs
- Antiarrhythmic agents
- Statins
- Connective tissue disease
- Systemic sclerosis
- Polymyositis
- Dermatomyositis
- Systemic lupus erythematosus
- Rheumatoid arthritis
- Infection
- Atypical pneumonia
- Pneumocystis pneumonia (PCP)
- Tuberculosis
- Chlamydia trachomatis
- Respiratory Syncytial Virus
- Interstitial Pneumonia: Bacteria, viruses, or fungi may infect the interstitium of the lung. A bacterium called Mycoplasma pneumonia is the most common cause.
- Idiopathic
- Sarcoidosis
- Idiopathic pulmonary fibrosis pneumonia (PCP)
- Hamman-Rich syndrome
- Antisynthetase syndrome
- Malignancy
- Lymphangitic carcinomatosis
Diagnosis of ILD
Patients with pneumocystis pneumonia can present with ILD, as seen in the reticular markings on this AP chest x-ray.
Investigation is customized towards the symptoms and signs. People with interstitial lung disease usually come to see a doctor due to concern about shortness of breath or cough. A proper and detailed history looking for the occupational exposure, and for signs of conditions listed above is the first and probably the most important part of the workup in patients with interstitial lung disease.
Interstitial lung diseases can be classified according to radiologic patterns
Pattern of opacities
- Consolidation
- Linear or reticular opacities
- Small nodules
- Cystic airspaces
- Ground glass opacities
- Thickened alveolar septa Associated findings:
- Pleural effusion or thickening
- Lymphadenopathy
Lung Biopsy
A lung biopsy is required if the clinical history and imaging are not clearly suggestive of a specific diagnosis or malignancy cannot otherwise be ruled out. A surgical lung biopsy is often required.
A lung biopsy is required if the clinical history and imaging are not clearly suggestive of a specific diagnosis or malignancy cannot otherwise be ruled out. A surgical lung biopsy is often required.
Patients with pneumocystis pneumonia can present with ILD, as seen in the reticular markings on this AP chest x-ray.
Investigation is customized towards the symptoms and signs. People with interstitial lung disease usually come to see a doctor due to concern about shortness of breath or cough. A proper and detailed history looking for the occupational exposure, and for signs of conditions listed above is the first and probably the most important part of the workup in patients with interstitial lung disease.
Pulmonary function tests
These usually show a restrictive defectwith decreased diffusion capacity (DLCO). X-rays
- Chest radiography is usually the first test to detect interstitial lung diseases, but the chest radiograph can be normal in up to 10% of patients.
- High resolution CT of the chest is the preferred modality
- Radiologic appearance alone however is not adequate and should be interpreted in the clinical context, keeping in mind the temporal profile of the disease process.
Treatment
ILD is not a single disease, but encompasses many different pathological processes. Hence treatment is different for each disease.
- If a specific occupational exposure cause is found, the person should avoid that environment. If a drug cause is suspected, that drug should be discontinued.
- Many cases due to unknown or connective tissue-based causes are treated with corticosteroids, such as prednisolone. Some people respond to immunosuppressant treatment
- Esbriet (pirfenidone) and Ofev (nintedanib): These drugs are FDA-approved to treat idiopathic pulmonary fibrosis. They act on multiple pathways that may be involved in the scarring of lung tissue. Studies show both medications slow decline in patients when measured by breathing tests.
- Patients with hypoxemia may be given supplemental oxygen.
- Pulmonary rehabilitation appears to be useful.
- Lung transplantation is an option if the ILD progresses despite therapy in appropriately selected patients with no other contraindications.
To study a case study regarding the successful treatment of ILD by homoeopathy, CLICK HERE
