Granulomatosis with polyangiitis medical therapy: Difference between revisions
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{{Wegener's granulomatosis}} | {{Wegener's granulomatosis}} | ||
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==Overview== | ==Overview== | ||
Revision as of 19:36, 10 April 2018
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Ali Poyan Mehr, M.D. [2]Associate Editor(s)-in-Chief: Krzysztof Wierzbicki M.D. [3]Cafer Zorkun, M.D., Ph.D. [4]Amandeep Singh M.D.[5]
Overview
In most cases, treatment consists of a combination of a glucocorticoid (a steroid) and a cytotoxic medicine. Although these medicines are helpful in treating Wegener's granulomatosis, people and their doctors should be aware that they potentially have serious side effects. In many instances these can be minimized or prevented by careful monitoring by both the doctor and patient.
Medical Therapy
Before steroid treatment became available, mortality within one year was over 90%, with average survival being 5 months. Steroids prolonged average survival to 8 months. The introduction of cyclophosphamide (CYC) in the 1970s was a major breakthrough.[1][2]
Initial treatment
Immunosuppressants
Glucocorticoids
- Parenteral regimen (pulse therapy)
- Preferred regimen (1):Methylprednisolone 7-15 mg/kg IV q24h for 3 days (maximum, 500-1000 mg/kg/day)
- Oral regimen (follows parenteral pulse therapy)
- Preferred regimen (1): Prednisone 1 mg/kg/day PO from Day 1 (maximum, 60-80 mg/day) tapered over 14 days with maximum of 20 mg/day
Cyclophosphamide
- Oral regimen
- Preferred regimen (1):Cyclophosphamide 1.5-2 mg/kg PO q24h for 3-6 months or till remission is achieved (Check CBC for Neutropenia and administer Mesna for hemorrhagic cystitis)
- Parenteral regimen (pulse therapy)
- Preferred regimen (1): Cyclophosphamide 15 mg/kg IV q2weekly for 3 doses and then q3weekly for 3-6 months (Check CBC for Neutropenia and administer Mesna for hemorrhagic cystitis)
- Patients using cyclophosphamide should also receive glucocorticoids.
Rituximab
- Parenteral regimen
Methotrexate
- Parenteral regimen
- Preferred regimen (1): Methotrexate 7.5 mg q1weekly
- . Once remission is attained (normally 3 to 6 months), treatment is frequently changed to azathioprine or methotrexate, which are less toxic drugs. Total duration of therapy should be at least one year, or longer in high risk patients. Corticosteroids are tapered to a low maintenance dose, 5-10 mg/day. Plasmapheresis may be beneficial in severe disease or pulmonary hemorrhage. Experience with other treatment agents is very limited.
Some guidelines for which drug to choose.[1]
- In localized disease, treatment with the antibiotic co-trimoxazole is recommended, with steroids in case of treatment failure.[3]
- In generalized non-organ threatening disease, remission can be induced with methotrexate and steroids, where the steroid dose is reduced after a remission has been achieved and methotrexate used as maintenance.
- In case of organ-threatening disease, pulsed intravenous cyclophosphamide with steroids is recommended. Once remission has been achieved, azathioprine and steroids can be used to maintain remission.
- In severe renal vasculitis, the same regimen is used but with the addition of plasma exchange.
- In pulmonary hemorrhage, high doses of cyclophosphamide with pulsed methylprednisolone may be used, or alternatively CYC, steroids, and plasma exchange.
In severe disease not responsive to previously mentioned treatment, the review is positive about mycophenolate mofetil, 15-deoxyspergualin, anti-thymocyte globulin, rituximab and infliximab; data was less favourable for intravenous immunoglobulin (IVIG) and etanercept.[1]
Methotrexate
Methotrexate has been studied at NIH for the treatment of Wegener's granulomatosis since 1990. In people with active, but not immediately life threatening, Wegener's granulomatosis, methotrexate has been used in combination with prednisone to bring about remission. It also is used to maintain remission after a person has initially received cyclophosphamide. Methotrexate is usually given for 1 to 2 years, after which time if people stay in remission, it is decreased and stopped.
Methotrexate is given once a week usually by mouth, but occasionally as an injection under the skin or in the muscle. People taking methotrexate need to have regular blood work to monitor their response and to watch for side effects.
The side effects of methotrexate include infection, lowering of the blood counts, nausea, soreness and ulceration of the mouth lining, irritation of the lungs (pneumonitis), and inflammation and scarring of the liver. People taking methotrexate cannot drink alcoholic beverages. Methotrexate cannot be given to people who have poor kidney function or who have underlying liver disease such as hepatitis.
Azathioprine
Azathioprine (also called Imuran) is used primarily to maintain remission in people who have initially been treated and gone into remission with cyclophosphamide. It is taken once a day by mouth. Similar to methotrexate, it is usually given for 1 to 2 years after which time the dosage is lowered until it is stopped.
The side effects of azathioprine include infection, lowering of the blood counts, and rarely an allergic type reaction. In people who receive azathioprine to prevent rejection of a transplanted organ, there has been a suggestion of an increased risk of blood cancers (leukemia and lymphoma) but it is not clear whether this risk exists in other situations. People with poor kidney function or liver disease can take azathioprine.
Other medicines
During the course of treating Wegener's granulomatosis, doctors often give their patients other medicines to prevent medicine-related side effects. These include
- Trimethoprim/sulfamethoxazole (also called bactrim or septra) is given three times a week to prevent Pneumocystis carinii infection (a lung infection)
- A medicine regimen is often given to prevent prednisone-related bone loss (osteoporosis)
- Folic acid or folinic acid (also called leucovorin) are often given to people taking methotrexate
References
- ↑ 1.0 1.1 1.2 Bosch X, Guilabert A, Espinosa G, Mirapeix E (2007). "Treatment of antineutrophil cytoplasmic antibody associated vasculitis: a systematic review". JAMA. 298 (6): 655–69. doi:10.1001/jama.298.6.655. PMID 17684188.
- ↑ Flossmann O, Berden A, de Groot K, Hagen C, Harper L, Heijl C, Höglund P, Jayne D, Luqmani R, Mahr A, Mukhtyar C, Pusey C, Rasmussen N, Stegeman C, Walsh M, Westman K (March 2011). "Long-term patient survival in ANCA-associated vasculitis". Ann. Rheum. Dis. 70 (3): 488–94. doi:10.1136/ard.2010.137778. PMID 21109517.
- ↑ Stegeman CA, Tervaert JW, de Jong PE, Kallenberg CG (1996). "Trimethoprim-sulfamethoxazole (co-trimoxazole) for the prevention of relapses of Wegener's granulomatosis. Dutch Co-Trimoxazole Wegener Study Group". N. Engl. J. Med. 335 (1): 16–20. PMID 8637536.