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Cryptogenic organizing pneumonia
Last reviewed: 23.04.2024
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Cryptogenic organizing pneumonia (bronchiolitis obliterans with organizing pneumonia) is an idiopathic lung disease in which the granulation tissue blocks the clearance of bronchioles and alveolar ducts, causing chronic inflammation and organizing pneumonia in adjacent alveoli.
Idiopathic obliterating bronchiolitis with organizing pneumonia (cryptogenic organizing pneumonia) affects men and women usually between the ages of 40 and 50 with the same frequency. Smoking is probably not a risk factor.
[Symptoms of cryptogenic organizing pneumonia
About half of patients present complaints reminiscent of those with community-acquired pneumonia (including a persisting influenza-like syndrome characterized by coughing, fever, malaise, fatigue, and weight loss). Progressive cough and shortness of breath during physical activity usually encourage the patient to seek medical help. In the physical examination, inspiratory rales are detected.
Diagnosis of cryptogenic organizing pneumonia
The diagnosis is established when analyzing anamnestic data, the results of a physical examination, radiation studies, lung function and histological examination of a biopsy material. Changes in radiography of chest organs are characterized by bilateral diffuse, localized in the peripheral regions by alveolar dimming at normal lung volumes; may also occur peripheral darkening, similar to that characteristic of chronic eosinophilic pneumonia. In rare cases, the alveolar darkening is one-sided. There are often recurrent and migratory infiltrates. Rarely at the onset of the disease, irregular linear or focal interstitial infiltrates or "honeycomb lung" can be observed. In HRCT, focal consolidation of air spaces, darkening by the type of frosted glass, nodal dimming of small sizes, thickening of the walls and dilation of the bronchi are revealed. Focal blackouts are more common in the peripheral parts of the lower lobes of the lungs. With CT, it is possible to detect a much more extensive lesion area than expected from chest radiography.
Pulmonary function tests usually detect violations of a restrictive type, although obstructive disorders ([FEV / FVC] <70%) occur in 21% of patients; in some cases, lung function does not suffer.
The results of the analyzes are nonspecific. Leukocytosis without increasing the number of eosinophils occurs in about half of the patients. Initially, ESR is often increased. Usually there is hypoxemia at rest and under stress.
Histological examination of the biopsy material of the lung tissue reveals a pronounced proliferation of granulation tissue in the small airways and alveolar ducts with chronic inflammation in the surrounding alveoli. Foci of organizing pneumonia (ie, changes characteristic of cryptogenic organizing pneumonia) are not specific and may be noted in other pathological processes, including infections, Wegener's granulomatosis, lymphomas, hypersensitivity pneumonitis and eosinophilic pneumonia.
Treatment of cryptogenic organizing pneumonia
Treatment of cryptogenic organizing pneumonia is similar to that of idiopathic pulmonary fibrosis. Clinical recovery is noted in two thirds of patients who received treatment, often within 2 weeks.
What is the prognosis of cryptogenic organizing pneumonia?
Cryptogenic organizing pneumonia has an unfavorable prognosis. Relapses occur in 50% of patients, but the effect of additional courses of glucocorticoids is characteristic.