Cryptogenic organizing pneumonia medical therapy
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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
The mainstay of the therapy is pharmacotherapy. 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 close monitoring, if there is worsening of symptoms or pulmonary function, 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 usually added to treatment.
Medical Therapy
- The mainstay of the therapy is pharmacotherapy.
- Corticosteroids are used as first-line treatment for patients with the symptomatic and progressive disease.
- For asymptomatic or nonprogressive disease, treatment is not required, observation is required till they become symptomatic.
Deciding factors to initiate medical therapy:
- Severity of symptoms.
- Pulmonary function test.
- The extent of disease on imaging.
- The rapidity of progression of symptoms.[1]
Standardized regimens of corticosteroids for the symptomatic and progressive disease are:
- Preferred regimen (1) Prednisone 0.75 mg/kg PO q24h for 4 weeks.
- Followed by (2) Prednisolone 0.5 mg/kg PO q24h for 4 weeks.
- Followed by (3) Prednisolone 20mg PO q24h for 4 weeks.
- Followed by (4) Prednisolone 10mg PO q24h for 6 weeks.
- Followed by (5) Prednisolone 5mg PO q24h for 6 weeks before they were stopped.
Treatment of cryptogenic organizing pneumonia according to the severity of disease:
Mild disease:
- Patient who have minimal symptoms, normal pulmonary function tests, and mild radiographic presentation.
- Treatment of mild disease is to monitor if there is no worsening of symptoms or pulmonary function.
- Patient is reassessed after 8 to 12 weeks for the worsening of symptoms and pulmonary function.
- For mild to moderate, macrolides (Clarithromycin 250 to 500 mg twice a day) are preferred by who don't want to use corticosteroids.
- Macrolides are used for 3 to 6 months and taper down to once daily.[2][3][4][5][6]
Persistent or gradually worsening disease:
- Patients have persistent severe progressing symptoms, moderate pulmonary function test impairment, and diffuse radiographic changes.
- According to British Thoracic Society guidelines, treatment of persistent disease is the initial dose of prednisone of 0.75 to 1 mg/kg per day, using ideal body weight, to a maximum of 100 mg/day given as a single oral dose in the morning. [1]
Severe cases:
- Preferred regimen (1)Prednisolone 2mg/kg IV q24h for first 3-5 days. Followed by the same regimen discussed above.
Failure to respond to systemic glucocorticoids:
- Patients have the stable disease but fail to improve with systemic glucocorticoids, then superimposed infection has to be excluded by repeating bronchoscopy with bronchoalveolar lavage.
- Treatment for persistent cryptogenic organizing pneumonia is to add cytotoxic agent with oral prednisone.
- Cytotoxic agent used is cyclophosphamide with the initial dose is 1 to 2 mg/kg per day up to a maximum of 150 mg/day.
Relapses:
- Relapses are very common with corticosteroids therapy.
- The predictors of relapses are:
- Delayed treatment.
- Increased gamma-glutamyltranspeptidase levels.
- Increased alkaline phosphatase levels.
Treatment of relapses:
- Azathioprine is used for the treatment of relapses.[10][11]
References
- ↑ 1.0 1.1 Bradley B, Branley HM, Egan JJ, Greaves MS, Hansell DM, Harrison NK, Hirani N, Hubbard R, Lake F, Millar AB, Wallace WA, Wells AU, Whyte MK, Wilsher ML (September 2008). "Interstitial lung disease guideline: the British Thoracic Society in collaboration with the Thoracic Society of Australia and New Zealand and the Irish Thoracic Society". Thorax. 63 Suppl 5: v1–58. doi:10.1136/thx.2008.101691. PMID 18757459.
- ↑ Epler GR, Colby TV, McLoud TC, Carrington CB, Gaensler EA (January 1985). "Bronchiolitis obliterans organizing pneumonia". N. Engl. J. Med. 312 (3): 152–8. doi:10.1056/NEJM198501173120304. PMID 3965933.
- ↑ Stover DE, Mangino D (November 2005). "Macrolides: a treatment alternative for bronchiolitis obliterans organizing pneumonia?". Chest. 128 (5): 3611–7. doi:10.1378/chest.128.5.3611. PMID 16304320.
- ↑ Ichikawa Y, Ninomiya H, Katsuki M, Hotta M, Tanaka M, Oizumi K (1993). "Low-dose/long-term erythromycin for treatment of bronchiolitis obliterans organizing pneumonia (BOOP)". Kurume Med J. 40 (2): 65–7. PMID 8231065.
- ↑ Vaz AP, Morais A, Melo N, Caetano Mota P, Souto Moura C, Amorim A (2011). "[Azithromycin as an adjuvant therapy in cryptogenic organizing pneumonia]". Rev Port Pneumol (in Portuguese). 17 (4): 186–9. doi:10.1016/j.rppneu.2011.03.010. PMID 21652172.
- ↑ Radzikowska E, Wiatr E, Gawryluk D, Langfort R, Bestry I, Chabowski M, Roszkowski K (2008). "[Organizing pneumonia--clarithromycin treatment]". Pneumonol Alergol Pol (in Polish). 76 (5): 334–9. PMID 19003763.
- ↑ Davison AG, Heard BE, McAllister WA, Turner-Warwick ME (1983). "Cryptogenic organizing pneumonitis". Q. J. Med. 52 (207): 382–94. PMID 6647749.
- ↑ Purcell IF, Bourke SJ, Marshall SM (March 1997). "Cyclophosphamide in severe steroid-resistant bronchiolitis obliterans organizing pneumonia". Respir Med. 91 (3): 175–7. PMID 9135858.
- ↑ Ning-Sheng L, Chun-Liang L, Ray-Sheng L (2004). "Bronchiolitis obliterans organizing pneumonia in a patient with Behçet's disease". Scand. J. Rheumatol. 33 (6): 437–40. PMID 15794207.
- ↑ Laszlo A, Espolio Y, Auckenthaler A, Michel JP, Janssens JP (March 2003). "Azathioprine and low-dose corticosteroids for the treatment of cryptogenic organizing pneumonia in an older patient". J Am Geriatr Soc. 51 (3): 433–4. PMID 12588596.
- ↑ Strobel ES, Bonnet RB, Werner P, Schaefer HE, Peter HH (1998). "Bronchiolitis obliterans organising pneumonia and primary biliary cirrhosis-like lung involvement in a patient with primary biliary cirrhosis". Clin. Rheumatol. 17 (3): 246–9. PMID 9694063.