Cryptogenic organizing pneumonia (patient information)

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Cryptogenic organizing pneumonia

Overview

What are the symptoms?

What are the causes?

Who is at highest risk?

Diagnosis

When to seek urgent medical care?

Treatment options

Where to find medical care for Asthma?

Prevention

What to expect (Outlook/Prognosis)?

Possible complications

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Manpreet Kaur, MD [2]

Overview

Cryptogenic organizing pneumonia is an idiopathic diffuse interstitial lung disease that affects the distal bronchioles, respiratory bronchioles, alveolar ducts, and alveolar walls. The injury occurs within the alveolar wall. Cryptogenic organizing pneumonia has no identifiable etiology. Some of the causes of organizing pneumonia include infectious agents, drugs, inflammatory diseases, and toxic exposures. The symptoms of cryptogenic organizing pneumonia usually develop in the fifth or sixth decade of life and start with symptoms such as a cough, dyspnea, and weight loss. Cryptogenic organizing pneumonia patients have the good prognosis after the course of corticosteroids. Fatal outcome of cryptogenic organizing pneumonia is very rare. Patients with cryptogenic organizing pneumonia usually appear in mild distress. On examination, the patient is usually febrile, in tachypnea, tachycardia with the regular pulse. On lung auscultation there are bilateral vesicular breath sounds, fine crackles and wheezing is heard. On a chest X-ray in cryptogenic organizing pneumonia findings like consolidation, which may be unilateral/ bilateral, migratory and affects all lung zone and foci of granulation tissue can also be seen. On CT scan findings are subpleural and peribronchovascularconsolidation, ground-glass attenuation and nodules, irregular linear opacities, bronchial wall thickening and dilatation and reverse halo sign (atoll sign). On pulmonary function testing, the restrictive pattern is seen mostly. Corticosteroids are used as first-line treatment for patients with the symptomatic and progressive disease. Treatment is planned according to the severity of the disease. For treatment of mild disease is close monitoring is required, if there is worsening of symptoms or pulmonary function, then macrolides are used in the treatment of mild disease.For persistent or gradually worsening disease, corticosteroids are used for treatment and for severe disease cytotoxic agents are added. Relapses are common with corticosteriods therapy, azathioprine is used. Surgical treatment is preferred for focal organizing pneumonia. For preventive measures, the patient should be educated about the risk factors and signs of worsening of the disease.

What are the symptoms of cryptogenic organizing pneumonia?

Patients with cryptogenic organizing pneumonia may have a positive history of connective tissue diseases, drug intake, malignancy and other interstitial lung disease. Common symptoms are dry cough, shortness of breath, fever, fatigue and weight loss. Less common symptoms are hemoptysis, chest pain and night sweats.

What causes of cryptogenic organizing pneumonia?

Some of the causes of organizing pneumonia include infectious agents, drugs, inflammatory diseases, and toxic exposures.

Who is at highest risk?

Cryptogenic organizing pneumonia affects men and women equally, usually beginning between the ages of 40 and 60.

Diagnosis

For diagnosis, history and physical examination is the first thing helps in diagnosing cryptogenic organizing pneumonia. Patients with cryptogenic organizing pneumonia usually appear in mild distress. On examination, the patient is usually febrile, in tachypnea, tachycardia with the regular pulse. On lung auscultation there are bilateral vesicular breath sounds, fine crackles and wheezing is heard. Following tests are ordered after examination:

When to seek urgent medical care?

When there is worsening shortness of breath, dry cough changed to a productive cough.

Treatment options

The mainstay of treatment is corticosteroids.

Where to find medical care for Cryptogenic organizing pneumonia?

Directions to Hospitals Treating Cryptogenic organizing pneumonia

Prevention

Education about the risk factors and need for early treatment is most useful preventive measure.

What to expect (Outlook/Prognosis)?

The overall prognosis for cryptogenic organizing pneumonia is significantly better than that of other interstitial lung diseases. Recovery occurs in approximately 2/3 of patients treated with steroids, with symptoms generally clearing over several months.

Possible complications

Fatal outcome of cryptogenic organizing pneumonia is very rare.

Sources

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