![]() (For more information on this disorder, choose “acute interstitial pneumonia” as your search term in the Rare Disease Database.)Ī diagnosis of OP may be made based upon a clinical evaluation, a detailed patient history, identification of characteristic findings and specialized tests such as x-ray studies, especially a high-resolution chest computed tomography (HRCT), pulmonary function studies that includes a diffusing capacity test and often a lung biopsy for microscopic tissue analysis. The name and its abbreviation, AIP, are limited to those cases of unknown cause. This pattern is almost identical to that found with acute respiratory distress syndrome (ARDS) and may be confused with it. ![]() (For more information on this disorder, choose “idiopathic pulmonary fibrosis” as your search term in the Rare Disease Database.)Īcute interstitial pneumonia (AIP) is an inflammatory lung disorder that progresses rapidly and is distinguished by the nature or pattern of cells found on biopsy of the lung. In this situation, the OP is secondary to the underlying IPF disorder, and treatment with prednisone may eliminate the OP, yet the underlying IPF continues. The OP process is sometimes seen in IPF as the inflammation component. Complications such as infection or heart problems may develop. When severe, the skin may appear slightly bluish (cyanotic) due to lack of circulating oxygen. Shortness of breath, mild at first and then severe is the major symptom. Idiopathic pulmonary fibrosis (IPF) is a fibrosing (scar-producing) and inflammatory lung disorder of unknown origin (idiopathic) characterized by abnormal formation of scar and fibrosis between the spherical, alveolar structures in the lung, which is referred to as the interstitium. Comparisons may be useful for a differential diagnosis. Symptoms of the following disorders can be similar to those of OP. This form of OP may be associated with an underlying fibrotic process. In rare cases affected individuals may experience chest pain, joint pain (arthralgia), night sweats or coughing up blood (hemoptysis).Ī rapidly progressive form of OP exists that can progress from symptom onset to acute respiratory failure in only a few days. Individuals with OP may develop small crackling or rattling sounds in the lung (crackles or rales) that are apparent upon physical examination. The shortness of breath and cough may become progressively worse. Eventually, shortness of breath, especially from exertional activities may develop. Some affected individuals develop a flu-like illness characterized by a sore throat, a general feeling of ill health (malaise), weight loss and fatigue. The most common symptom is a persistent, nonproductive cough. Symptoms usually develop slowly over a few weeks or months. Some individuals with OP such as focal OP may have no apparent symptoms, while others may have severe respiratory distress as in acute, rapidly progressive OP. For example, people with idiopathic OP have a flu-like illness, while people with OP associated with an underlying connective-tissue disorder have cough or shortness of breath. Symptoms of organizing pneumonia vary from person to person depending upon the specific type. Since the first description in 1980 as bronchiolitis obliterans organizing pneumonia (BOOP), OP has been known by different names. If the symptoms do not improve other treatment may be needed. When treatment is needed, corticosteroids, such as prednisone, are the most used medication. Some people affected with this condition do not need treatment. ![]() Idiopathic organizing pneumonia may also be called cryptogenic organizing pneumonia (COP). Several different known causes of OP have been identified, but most cases occur for no known reason (idiopathic). It is a different disease from obliterative bronchiolitis which refers to scarring and narrowing of the small airways. Though the term pneumonia is used, OP is not an infection. Individuals with OP experience inflammation of the bronchioles and alveolar lung spherical units simultaneously, which distinguishes it from other similar inflammatory lung disorders. OP refers to organized swirls of inflammatory tissue filling the small spherical units of the lungs called alveoli as well as the alveolar ducts. Wheezing and hemoptysis (blood when coughing) may occur rarely. Organizing pneumonia (OP) is a rare inflammatory lung disorder characterized by clinical symptoms such as flu-like illness as well as cough and shortness of breath with exertional activities. ![]()
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