Board of lung lung fibrosis specialist praxis medizinicum hamburg

Board of fibrosing lung diseasesFibrosing lung diseases are often grouped under the collective term pulmonary fibrosis. However, pulmonary fibrosis can have different causes.

Pulmonary fibrosis primarily affects the connective and supporting tie of the alveoli, the hair-thin pulmonary vessels and the small airways. The disease processes therefore take place in parts of the lung called the lung skeleton, lung parenchyma or interstitium. International linguistic usage therefore speaks of interstitial lung diseases (ILD), and interstitial pulmonary fibrosis (IPF).

The different diseases of the lung

The different diseases have known or unknown (idiopathic) causes. The most common disease is idiopathic pulmonary fibrosis (about 20%), which mainly affects the lungs. An additional 20% of patients have pulmonary involvement in the setting of inflammatory rheumatic diseases such as rheumatoid arthritis (inflammatory rheumatism of the joints), systemic lupus erythematosus (inflammatory soft tie rheumatism involving many organs), poly/dermatomyositis (muscle inflammation with or without skin involvement), systemic sclerosis (skin induration with or without involvement of internal organs), or microscopic polyangitis (inflammation of small blood vessels).

Diagnosing fibrosing lung disease as early as possible is of great importance, as the extent of lung involvement determines the severity of the disease and life expectancy (mortality). Due to the complexity of the disease, patients should always be accompanied by an interdisciplinary team of physicians.

Complaints of the lungs

Dry cough and shortness of breath during physical exertion are the first and most common symptoms, which increase as the lung disease progresses.

Diagnosis of pulmonary fibrosis

The physician's detailed medical history concerns the extent of the symptoms as well as possible risk factors such as exposure to pollutants in the private and professional environment. Furthermore, a search is made for any underlying diseases that may be present, for example rheumatic diseases. During the physical examination, the lungs should always be examined with the aid of a stethoscope. Fine-bubble rales on deep inspiration are a characteristic feature of pulmonary fibrosis.

Pulmonary function testing

Pulmonary fibrosis requiring treatment is always accompanied by a reduction in lung function. Detailed pulmonary function testing with spirometry and whole-body pletysmography objectifies the restriction of lung volumes (restrictive ventilation disorder), which is a characteristic finding in advanced interstitial lung disease. Measurement of gas exchange (diffusion capacity) of the lung describes the ability to transport oxygen into the pulmonary vessels and to release carbon dioxide. This vital process is also hindered by pulmonary fibrosis. Consequently, blood gases are also abnormally altered. Should therefore be measured at rest as well as during exercise (spiroergometry).

CT lung examination

The different forms of pulmonary fibrosis lead to characteristic morphological changes in the lungs, which can be detected by means of high-resolution computed tomography (CT). Since the cause of some pulmonary fibrosis diseases is not known, the patterns of the CT scan help to facilitate the classification of these clinical pictures. The most common idiopathic pulmonary fibrosis is associated with a radiographic pattern known as usual interstitial pneumonia UIP. UIP is therefore not a diagnosis, but a fine tie or radiological feature of a disease. Another important pattern is non-specific interstitial pneumonia (NSIP), which is found particularly in lung involvement in the setting of rheumatic inflammatory diseases.

Endoscopic and surgical diagnostics

Not all fibrosing diseases of the pulmonary skeleton can be clearly identified by clinical, functional, and radiologic examinations. Therefore, endoscopic (bronchoscopy) and surgical procedures may be necessary to confirm the diagnosis.

Endoscopy of the airways with the aid of a flexible bronchoscope can be performed on an outpatient basis under local anesthesia. Bronchoscopy allows bronchoalveolar lavage (BAL) to be performed. The immune cells present on the inner surface of the alveoli are obtained by lavage, providing information about the inflammatory and fibrotic tie changes. Bronchoscopy also allows tie samples to be taken under appropriate conditions.

If all previous procedures do not lead to a diagnosis, surgical removal of lung tie may be considered. The risk of such a procedure must be weighed against the benefit of histopathologic examination of lung tie.

Interstitial lung disease in rheumatic diseases

With the help of the previously mentioned examination methods and clinical observation, it was possible to work out important differences between usual interstitial pneumonia (UIP) in idiolathic interstitial lung disease and non-specific interstitial pneumonia (NSIP), which occurs primarily in rheumatic inflammatory diseases. UIP is primarily a fibrosing disease in which the normal lung tie is replaced by fiber-rich connective tie; inflammatory processes play only a minor role here. In contrast, early-stage NSIP is inflammation that only develops into fibrosis later in its course. If the inflammation is detected early and treated with anti-inflammatory agents, the progression of fibrosis can be halted or, ideally, completely prevented.

The positive effects of nintedanib on the progression of IPF has led to the use of nintedanib in other fibrosing lung diseases that do not have the UIP pattern of IPF. A double-blind, randomized, placebo-controlled study was conducted in 15 countries and on 31. October 2019 published in the New England Journal of Medicine. The results showed that patients with progressive fibrosing lung disease benefited from treatment with nintedanib regardless of the radiographic pattern of lung changes. The annual rate of worsening forced vital capacity was significantly lower in patients treated with nintedanib compared with placebo. This study demonstrates that antifibrotic therapy should be used for all forms of pulmonary fibrosis, provided there is evidence of progressive deterioration. In non-specific interstitial pneumonia, the distinction between the early inflammatory phase has. The late stage characterized by fibrosis is of great importance in choosing the appropriate therapy. It is now well established that the drugs used in the Early stage NSIP dominated by inflammation can be treated effectively by an anti-inflammatory therapy (Immunosuppression). The earlier such therapy is given, the better the treatment results. In contrast, anti-inflammatory immunosuppression is largely ineffective in advanced disease with exclusive fibrosis. While initially intensive immunosuppression with cyclophosphamide was the first-line therapy, more recently the less intensive and often better tolerated mycophenolate mofetil has been used with similar effectiveness. Studies on several other anti-inflammatory drugs have been started. Exclusive cortisone therapy does not produce good long-term results in this disease and is considered outdated.

As previously outlined, two new drugs, pirfenidone and nintedanib, are available that do not primarily target inflammation but rather the Fibrose act. In parallel with the Nintendanib study in idiopathic pulmonary fibrosis mentioned above, another large study was published in May 2019 showing that Nintedanib also significantly slowed the worsening of lung disease due to scleroderma. Nintedanib is the first drug for which such an effect in a rheumatic disease is well established. A major study of pirfenidone in this situation has begun.

Like this post? Please share to your friends:
Leave a Reply

;-) :| :x :twisted: :smile: :shock: :sad: :roll: :razz: :oops: :o :mrgreen: :lol: :idea: :grin: :evil: :cry: :cool: :arrow: :???: :?: :!: