How To Cope With Interstitial Lung Disease?

How To Cope With Interstitial Lung Disease?

Early referral to the specialist allows diagnosis and rapid treatment to optimize the results and improve prognosis for interstitial lung disease. This process can be facilitated by community investigations, including chest radiograph and some serological studies (blood tests).

The control of interstitial lung disease symptoms, occupational therapy, and palliative care are also important aspects of patient management and coping.

Below is brief information on the coping of specific interstitial lung disease:

Idiopathic Pulmonary Fibrosis (IPF)

Previous treatments such as steroids, cyclophosphamide, triple treatment (prednisolone, azathioprine, and N-acetylcysteine), or just N-acetylcysteine have been shown to be ineffective or dangerous, so these options are no longer recommended. However, N-acetylcysteine and other mucolytics are sometimes used as expectorants.

The main side effect is diarrhea, anticoagulants are contraindicated and it is necessary to monitor lung function. Other drugs are in phase 2b of the research (simtuzumab, lebrikizumab), which raises hopes for the future clinical treatment of IPF.

Connective Tissue Disorder Associated With Interstitial Lung Disease

The involvement of the lung in connective tissue diseases ranges from fibrosis to pulmonary vascular disease, vasculitis, and pleural complications. Despite the considerable burden of pulmonary complications of connective tissue disease, the treatment of interstitial lung disease is not supported by convincing evidence. The only randomized controlled trials refer to the use of cyclophosphamide in scleroderma. One of them showed a modest significant effect on lung function and quality of life related to health.

The agents of choice are immunosuppressants. Despite its risks, the basic evidence for its use is minimal, with few data to guide the initiation and duration of optimal interstitial lung disease treatment.

Sarcoidosis

Sarcoidosis is a multisystem, granulomatous, inflammatory condition that typically occurs in younger adults, and extrathoracic symptoms such as erythema nodosum are not uncommon. Patients can go from stage 1 of the interstitial lung disease (only bihilar lymphadenopathy) to stage 2 or more (involvement of the lung parenchyma) without knowing that they are affected by sarcoidosis. Up to 10% can progress to fibrosis.

Hypersensitivity Pneumonitis

Hypersensitivity pneumonitis (extrinsic allergic alveolitis) can occur at any age and is due to the exaggerated immune response to the inhalation of antigens.

The management includes the identification and elimination of the sensitizing agent, which may be obvious but is usually difficult to identify and even more difficult is to eliminate the allergens, especially the ubiquitous environmental agents. Often, when the possible allergen cannot be counteracted or eliminated and when there is functional deterioration, the symptoms are controlled by steroids. There are no controlled works on the management of chronic hypersensitivity pneumonitis. Intravenous cyclophosphamide can be effective and there are case reports with good results using biological agents for refractory cases.

Pulmonary Rehabilitation

There is sufficient evidence that pulmonary rehabilitation helps patients with dyspnea. Unfortunately, the researchers say, pulmonary rehabilitation is not universally available in many regions and is restricted to patients with Chronic Obstructive Pulmonary Disease (COPD), in whom the effects are better known and well documented. The benefits are seen in the improvement of the strength of non-respiratory musculature. It can also be offered psychosocial support, nutritional advice and recommend abandoning the habit of smoking.

Lung Transplant

Lung transplantation improves interstitial lung disease survival in very well selected cases. Its purpose is to improve survival and quality of life; the prognosis of survival without a transplant is <50% at 5 years. 23% of lung transplants are performed in patients with idiopathic pulmonary fibrosis (IPF). Connective tissue diseases comprise <4% of their indication, while in other interstitial pulmonary diseases the percentage is lower.

What Are The Considerations For End-of-Life Care?

The coping of these patients encompasses much attention at the end of life. Some patients present themselves too late to do anti-fibrotic treatment, and all that can be done is to provide support for the management of the symptoms. It is important to consider the patient’s expectations from the beginning, even those in which a specific treatment is indicated since the effects of the treatments are still scarce in most of them.

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