<unk>  treatment depends on its severity and whether it has caused organ damage. Severe disease The standard treatment for severe<unk>  is to induce remission with immunosuppressants such as rituximab or cyclophosphamide in combination with high-dose corticosteroids. Plasmapheresis is sometimes recommended for very severe manifestations of<unk> , such as diffuse alveolar hemorrhage and rapidly progressive glomerulonephritis (as seen in pulmonary-renal syndrome). The use of plasmapheresis in those with<unk>  and acute kidney failure (renal vasculitis) might reduce progression to end-stage kidney disease at three months. Oral and intravenous cyclophosphamide are both effective for induction of<unk>  remission. Oral cyclophosphamide at a dose of 2 mg/kg/day was the standard treatment for many years; this regimen resulted in complete remission in more than 75% of people with<unk>  but is associated with significant toxicities including infertility, inflammation and bleeding from the bladder, and bladder cancer. In contrast, administering pulsed doses of intravenous cyclophosphamide is equally effective for inducing remission, results in a lower cumulative dose, and decreases the incidence of abnormally low white blood cell counts by one-third. However, pulsed intravenous cyclophosphamide may be associated with a higher risk of<unk>  relapse when compared to oral cyclophosphamide. Due to a high frequency of abnormally low white blood cell counts seen with cyclophosphamide treatment, Pneumocystis jirovecii pneumonia is a common complication and prophylaxis against this pathogen is recommended. Rituximab may be substituted for cyclophosphamide to induce remission since it is similarly effective and has a comparable side effect profile. The dose of corticosteroids is generally tapered (decreased) very slowly over the course of several months to reduce the risk of another<unk>  flare. After a person with<unk>  has successfully undergone induction and gone into remission, the treatment goal then shifts to maintenance of remission and preventing subsequent<unk>  flares. Less toxic immunosuppressing medications such as rituximab, methotrexate, azathioprine, leflunomide, or mycophenolate mofetil are used. TNF inhibitors, such as etanercept, appear to be ineffective and are not recommended for routine use. Limited disease In generalized non-organ-threatening disease, remission can be achieved with a combination of methotrexate and corticosteroids, where the steroid dose is reduced after a remission has been achieved and methotrexate is used as maintenance therapy. Treatment measures for localised<unk>  of the nose and sinuses includes nasal irrigation, nasal corticosteroids, and antibiotics if infection occurs. If perforation of the nasal septum occurs (or saddle nose deformity), then surgical repair is recommended. Trimethoprim/sulfamethoxazole has been proposed to help prevent relapse though a 2015 Cochrane review did not confirm fewer relapses with trimethoprim/sulfamethoxazole treatment.
Granulomatosis with polyangiitis
Microscopic polyangiitis
Polyarteritis nodosa
Thromboangiitis obliterans
Eosinophilic granulomatosis with polyangiitis
Lupus vasculitis
Nephrogenic systemic fibrosis
Transplant glomerulopathy