![]() reported a cohort of 1,346 patients with OP, among which 176 had COP and 1,170 had SOP ( 40). The number of patients with OP described in previous reports varies from a few cases to 48 in the study by Lazor et al., 66 patients with COP in the study by Radzikowska et al., 76 patients with COP in the study by Yoo et al., and 100 patients in the study by Yilmaz et al. According to a Greek registry, the COP prevalence is approximately 5% among patients with interstitial lung disease a similar registry in Spain indicates that approximately 10% of patients with interstitial lung disease also have COP ( 26, 27). Approximately 3% of patients with interstitial lung disease are presumed to have a diagnosis of OP ( 5, 6). The incidence of COP has been decreasing in recent years because of improvements in the diagnosis of causative factors. ![]() An Icelandic study revealed incidences of 1.10/100000 for COP and 0.87/100000 for SOP ( 26). The incidence of OP differs among populations it is estimated to be 1.97–7/100000 ( 3, 5, 6). The epidemiology of OP is poorly documented. The disease is classified into the following forms according to the course and nature of lesions detected during histological examination: OP, acute fibrinous organizing pneumonia (AFOP), and cicatricial variant of organizing pneumonia (CIOP) ( 1, 5, 6, 19– 25).įocal OP is a specific form that usually progresses asymptomatically lesion resection is sufficient treatment in many cases ( 3, 5, 6). In the era of administering steroid-sparing therapies for the treatment of interstitial lung diseases, connective tissue dases, and other conditions, it is important to emphasize this type of therapy for patients with COP. Several specific forms of OP (e.g., the cicatricial variant or acute fibrinous type) have distinct clinical and histological features, require higher doses of immunosuppressive drugs, and have a worse prognosis. Patients maintain sufficient respiratory function, and treatment with steroids is usually effective. The classical form of COP is usually subacute and does not manifest as severe disease. New biological therapies, interferon, monoclonal antibodies, anti-interleukin antibodies, and PD1/PDL-1 inhibitors may induce this specific pulmonary reaction. There has been an increase in the number of reports of drug-induced organizing pneumonia (OP). Secondary organizing pneumonia is diagnosed when the triggering factor has been identified it is mainly caused by infections, toxic substance exposure, drugs, connective tissue diseases, malignancies, autoimmune diseases, bone marrow, or organ transplantation, and radiotherapy. III Department of Lung Diseases and Oncology, National Tuberculosis and Lung Diseases Research Institute, Warsaw, PolandĬryptogenic organizing pneumonia (COP) is a form of idiopathic interstitial pneumonia that results from the pulmonary reaction to various unidentified injuries.A reticular roentgenographic pattern, fibrotic changes mainly in subpleural location at CT, lack of lymphocytosis in BAL fluid and histological features of usual interstitial pneumonitis and BOOP in biopsy specimens are all considered risk factors for a fatal outcome.Elzbieta Radzikowska * † Justyna Fijolek † CT can show the pattern of ground glass opacities, areas of airspace consolidation and small, round opacities, while considerable fibrotic change is usually not seen. In some cases small, round opacities may be observed as the only abnormality. Radiographically, BOOP is characterized by patchy areas of consolidation that are usually bilateral, peripheral and often migratory. Chest radiography, computed tomography (CT), bronchoalveolar lavage (BAL) and open lung biopsy are the primary diagnostic techniques. Early diagnosis and therapy are mandatory. A subset of BOOP, however, is associated with a fulminant course and a high lethality due to respiratory failure. The disease usually presents as a subacute illness with complete resolution. Histopathologically, it is characterized by plugs of fibroplastic connective tissue within respiratory bronchioles, mononuclear cell infiltrates in parenchyma adjacent to the affected bronchioles and foam cells within alveolar air-spaces. Bronchiolitis obliterans with organizing pneumonia (BOOP) is a clinicopathological syndrome of unknown etiology.
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