Bronchiolitis Obliterans Organising Pneumonia H014

Current RMA Instruments

Changes from previous Instruments

ICD Coding

• ICD-9-CM Code: 516.8
• ICD-10-AM Code: J84.0

Brief description

This SOP covers some forms of organising pneumonia, including idiopathic BOOP and some secondary (known cause) forms of organising pneumonia.  The condition may be a temporary phase which may resolve or change into another respiratory pathology.

Confirming the diagnosis

This is a complex diagnosis requiring specialist opinion.  Lung histology is needed for a definitive diagnosis, but the diagnosis can also be confirmed for DVA purposes based on typical clinical presentation and findings on high resolution CT scanning.  Other investigations, including pulmonary function tests and flexible bronchoscopy with bronchoalveolar lavage may also be undertaken. CMA advice should be obtained to confirm that the diagnosed condition is covered and not excluded by the SOP.

The appropriate medical specialist is a respiratory physician.

Additional diagnoses covered by SOP

• Cryptogenic organising pneumonia (COP)
• Idiopathic bronchiolitis obliterans organising pneumonia
• Organising pneumonia secondary to drugs
• Organising pneumonia secondary to ionising radiation
• Organising pneumonia secondary to local bronchial obstruction
• Organising pneumonia secondary to toxic fumes

Conditions excluded from SOP

• Acute Respiratory Distress Syndrome^#
• Asbestosis*
• Bronchiolitis obliterans (i.e. without organising pneumonia)
• Chronic bronchitis and emphysema*
• Chronic Obstructive Airways Disease / Chronic Obstructive Pulmonary Disease
• Fibrosing interstitial lung disease*
• Organising pneumonia as a manifestation of systemic disease such as malignant neoplasia; systemic inflammatory condition; or autoimmune connective tissue diseases. Examples are Sarcoidosis (SOP); Systemic Lupus Erythematosus (SOP); Rheumatoid arthritis (SOP); Inflammatory bowel disease
• Organising pneumonia due to infection. That is viral pneumonia, bacterial pneumonia, fungal pneumonia, or parasitic pneumonia^#
• Organising pneumonia that occurs in the presence of another interstitial lung disease and is not the dominant histological finding
• Pulmonary inflammation that is confined to the bronchioles (obliterative bronchiolitis, proliferative bronchiolitis)^#

* another SOP applies

^# non-SOP condition

Clinical onset

Onset is typically in the fifth or sixth decades of life.  The condition typically presents with dry cough, shortness of breath, fever, and malaise that are of relatively short duration (weeks to months).

Clinical worsening

The normal course of the condition varies with the underlying cause and with severity at time of presentation.  If exposure to the inciting agent can be stopped (e.g. a drug or irritant) then the condition can improve quite quickly. Patients with the cryptogenic form usually have persistent and progressive symptoms.  With appropriate treatment the outcome is variable, ranging from a complete response in the majority, through to no response and a fatal outcome in a small percentage.