Background Between 1988 and 2000 14 patients affected by CSS have been followed. Our series included 9 females and 5 males, the mean age at the diagnosis was 54 years (range 21–78), the mean follow up period was 95 months (range 60–141). Four criteria were simultaneneously satisfied in all patients at the diagnosis, according to the vasculitis ACR classification. The most important clinical manifestations exept for asthma, eosinophilia and paranasal sinus abnormality costantly present, were arthralgias, cutaneous nodules, peripheral neuropathy. Renal and gastroinestinal disease was observed only in one patient, cardiovascular and central nervous system involvement was respectively seen in 2 and 3 cases. Out of the 5 patients tested for ANCA, 2 resulted positive; prednisone treatment was started in 11 of the 14 patients at the mean dosage of 1 mg/kg/day, whereas at the 3 remaining patients was associated Cyclophosphamide at the dosage of 2 mg/kg/day. Only in one case IVIG pulse at the dosage of 1 gr/kg/for 5 days was started with good result.
The most recurrent side effects were urinary infections (3 cases), pulmonary infections (2 cases), tubercolosis (2 cases), diabetis (2 cases), Herpes Zoster (3 cases). Remissions were observed in all patients, relapses were seen though well controlled by the increase of the steroid therapy. 3 patients died: 1 for mesenteric vasculitis, 1 for respiratory insufficiency and the last one for reasons not correlated to the disease. As reported in letterature, also in our experience survival is influenced by the systemic involvement of the disease and by FFS (Five factors score: combination of renal, cardiac, neurologic, gastrointestinal involvement plus proteinuria). In our series survival at 120 months was 75%, the worst prognostic index was seen in patients with FFS > 1.
Statistics from Altmetric.com
If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.