Cop J84.9

The disease was first described in 1983 by Davison et al.

Acute/subacute interstitial lung disease (ILD), also known as cryptogenic organizing pneumonia (COP) according to the classification of the ATS (American Thoracic Society)

Here there are different indications in the literature. Some quantify the occurrence of the COP as between the 5th - 6th decade of life, others speak of the occurrence between the 20th and 80th year of life. Men and women are equally affected. Non-smokers and ex-smokers are affected twice as often as smokers!

The COP often occurs idiopathically. It is rare and accounts for only about 3% of all interstitial lung diseases (IIPs). Together withacute interstitial pneumonia(AIP), COP is one of the acute or sub-acute IIPs.

• Cough
• Fever
• Feeling ill
• Tiredness
• Weight loss

Pulmonary function: Most often there is a restriction and arterial hypoxemia.

X-ray: Bilateral alveolar shadows, partly spotted, partly diffusely distributed with preserved lung volume. Often there are recurrent and also migrating shadows.

HRCT: In HRCT there are areas of consolidation, nodular shading, milky glass like condensation and thickening or dilatation of the bronchial wall.

The "tree-in-bud phenomenon" is characteristic of the COP: In the subfields and periphery, nodular densifications of branching centrilobular structures similar to a flowering branch are found. In about 30% of the cases there are pleural effusions and an enlargement of the hilus lymph nodes.

• BSG increased
• C-reactive protein increased
• Neutrophilia

In lung biopsy, granulation tissue is found within the alveoli and alveolar ducts. In addition, there is a chronic inflammation in the form of pneumonia foci in the alveoli.

Any kind of tissue removal - sometimes even the BAL - can trigger an acute flare of disease in IPF/UIP. This is not described for the other subgroups. For this reason, however, the indication for biopsy should be narrowly defined.

• Bronchial carcinoma
• Cryptococcosis
• Granulomatosis
• Lymphoma
• exogenous allergic alveolitis
• Eosinophilic pneumonia

Complications

• respiratory insufficiency
• pulmonary hypertension
• cor pulmonale

In 2/3 of the patients a clinical recovery is achieved under glucocorticoids.

However, it is also possible that the disease - despite the glucocorticoids - takes a rapidly progressive fatal course.

Prednisolone: Both the optimal dosage and the duration of treatment are not known in interstitial lung diseases. The following schemes can be found in the literature:

1. Initial dose 0.5 - 1 mg/kg bw/d orally, but not more than 50 mg/d. This treatment should be carried out for 4-12 weeks and the patient should then be re-examined in detail.
2. Initial dose 1 mg/kg bw/d, but not more than 50 mg/d. After 4 weeks halve the dose, after another 4 weeks halve it again and examine the patient in detail.

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