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Few randomized clinical trials (RCTs) have been performed in patients with Churg-Strauss syndrome. Therefore, it is recommended to treat patients with this syndrome in accordance with the current practice guidelines for therapy for patients with ANCA-associated vasculitis. Standard therapy is prednisolone in combination with cyclophosphamide. Cyclophosphamide is stopped 3-6 months after diagnosis and subsequently azathioprine is started. Recently, it was demonstrated in two RCTs that B cell depletion by rituximab can be used as therapy for moderate to severe ANCA-associated vasculitis. Remission rates and adverse events were comparable with those of treatment with cyclophosphamide. It is pivotal to start therapy in patients with Churg-Strauss syndrome as soon as possible after the diagnosis is made.

A 55-year-old man, with no previous history, presented with extreme fatigue and fever and was admitted to hospital. He had progressive renal dysfunction and his serum anti-neutrophil cytoplasmic antibodies (ANCA) were markedly elevated. Renal histology was consistent with ANCA-associated vasculitis. The patient was successfully treated with cyclophosphamide and prednisolone. The classification and management of the ANCA-associated vasculitides are described. The classification was guided by the clinical presentation, serology and results of tissue biopsies. The ANCA inflammation had affected the middle sized and small vessels of especially the upper and lower airways, and the kidneys. The antibodies were directed at proteinase-3 (PR3) or myeloperoxidase (MPO). PR3-ANCA is predominantly found in Wegener's granulomatosis, while MPO-ANCA is related to microscopic polyangiitis. Tissue studies showed granulomatous inflammation of the airways which is typical of Wegener's disease. This type of inflammation is absent in microscopic polyangiitis. The initial treatment schedule consists of prednisone 1 mg/kg daily and oral cyclophosphamide 2 mg/kg daily. In the remission phase, the cyclophosphamide is replaced by azathioprine. It is not yet known how long maintenance treatment should be continued and which parameters have prognostic value.

Standard therapy of Wegener's granulomatosis is prednisolone in combination with cyclophosphamide. Cyclophosphamide is continued for 15-18 months after diagnosis. During therapy 26% of the patients have severe or life-threatening side effects, mostly infections. Furthermore, there are multiple long-term side effects such as an increased incidence of malignancies, especially bladder carcinoma. During the last 10 years the European vasculitis study group (www.vasculitis.org) organized several randomized clinical trials to determine optimal treatment. Recently, it was demonstrated in one of these trials that in patients with generalized ANCA-associated vasculitis (vasculitis associated with antineutrophil cytoplasmic autoantibodies) withdrawal of cyclophosphamide and substitution by azathioprine after remission did not increase the rate of relapse and was safe. Wegner's granulomatosis is not a fatal disease anymore. Sometimes, however, patients lose their job. Fortunately, this occurs only in a small percentage of patients.

Various forms of vasculitis may result in mesenteric ischaemia, ischaemic colitis or aneurysm formation in the aorta or intestinal blood vessels. Vasculitides may involve large- and/or medium-sized vessels, medium- and/or small-sized vessels, or small-sized vessels only. It is essential to differentiate between the different forms of vasculitis since diagnostic tests and therapies differ greatly. Gastrointestinal manifestations of vasculitis can generally be detected using angiography, digital subtraction angiography and/or magnetic resonance angiography (MRA). Various laboratory tests are helpful in establishing the diagnosis in patients in whom vasculitis is clinically suspected. In addition, the diagnosis should be confirmed using histology or angiography if possible. Treatment of vasculitis not caused by chronic infection consists of high dose corticosteroids and, in the case of polyarteritis nodosa or vasculitis associated with anti-neutrophil cytoplasmic antibodies (ANCA), cyclophosphamide.

A 20-year-old woman presented with abdominal pain, purpura on the extremities, and arthralgia. The diagnosis of Henoch-Schönlein purpura was made based on granular IgA deposits in the vessels in a skin biopsy. Three weeks after onset of these symptoms, she developed glomerulonephritis and diffuse alveolar haemorrhage; she developed respiratory failure and needed mechanical ventilation. She recovered upon treatment with corticosteroids. Diffuse alveolar bleeding has occasionally been reported in adults with Henoch-Schönlein purpura; this is the first report from the Netherlands.

A diagnosis of vasculitis generally requires histopathological proof of vasculitis, particularly because many other disease entities may mimic vasculitis ('vasculitis-look-alikes'). Vasculitis can be primary (idiopathic) or secondary to other diseases, especially infectious diseases. The primary vasculitides are classified according to the size of the vessels involved and the nature of the inflammation, in combination with clinical symptoms. The detection of autoantibodies, particularly the antineutrophil cytoplasmic antibodies (ANCA), in some of the primary vasculitides, has facilitated the diagnosis of those diseases and has given more insight into their pathophysiology. Treatment of primary vasculitis still consists of corticosteroids whether or not in combination with immunosuppression. New insights in pathophysiology may provide more specific and less toxic treatment modalities.