Necrotizing vasculitis in Greece: clinical, immunological and immunogenetic aspects. A study of 66 patients
| Autor: | Urania Dafni, C A Papasteriades, H. M. Moutsopoulos, Kyriaki A. Boki, Alexandros A. Drosos, George Karpouzas |
|---|---|
| Rok vydání: | 1997 |
| Předmět: |
Adult
Male Vasculitis medicine.medical_specialty Pathology C-ANCA Systemic disease Adolescent Churg-Strauss Syndrome Gastroenterology Antibodies Antineutrophil Cytoplasmic Autoimmune Diseases Necrosis Rheumatology HLA Antigens Internal medicine Weight Loss Necrotizing Vasculitis medicine Humans Pharmacology (medical) Cyclophosphamide Survival rate Aged Retrospective Studies Hepatitis B Surface Antigens Greece business.industry Vascular disease Polyarteritis nodosa Incidence Granulomatosis with Polyangiitis Middle Aged medicine.disease Polyarteritis Nodosa Survival Rate Treatment Outcome Antirheumatic Agents Disease Progression Female Microscopic polyangiitis business |
| Zdroj: | Rheumatology. 36:1059-1066 |
| ISSN: | 1462-0332 1462-0324 |
| DOI: | 10.1093/rheumatology/36.10.1059 |
| Popis: | SUMMARY The clinical spectrum and outcome of necrotizing vasculitis were evaluated in a retrospective study in order to assess: (1) the clinical expression and evolution of the disease; (2) the response to several therapeutic approaches based on major events (organ involvement causing disability or death); (3) the immunogenetic background of patients. Sixty-six Greek patients fulfilling the ACR criteria for the diagnosis of vasculitis entered the study. Thirty-seven were diagnosed with Wegener’s granulomatosis (WG), 22 with polyarteritis nodosa (PAN) and seven with Churg‐Strauss syndrome (CSS). The demographic characteristics of patients with WG and PAN were similar. Cutaneous manifestations, gastrointestinal and peripheral nervous system involvement occurred more often in patients with PAN, whereas pulmonary and upper respiratory tract involvement, renal, ear abnormalities and fever were more frequent in patients with WG. Muscle weakness and asthma were found exclusively in patients with PAN and CSS, respectively, while the presence of classic-antineutrophil cytoplasmic antibodies (cANCA) characterized WG patients. Hepatitis B surface antigen (HBsAg) was found in 22% of PAN patients. No significant diAerences were detected when comparing the PAN and WG groups with respect to the first major event (log-rank P = 0.50) with and without potential confounders (age, gender, therapy or c-ANCA). For WG patients, a statistically significant diAerence was found on diAerent routes of administration of cyclophosphamide (oral vs pulse) (P = 0.006). Regarding the HLA antigens, an increased frequency of DR1 (26.9% vs 10.3%, P = 0.057) in WG and the absence of DR3 in patients with PAN and CSS were noted. It appears that although the immunogenetic background and the clinical expression of the diseases diAer, the response to treatment as well as the evolution and the survival rate of these patients are similar in the two groups. VASCULITIS is a clinicopathological entity characterized by inflammation and necrosis of blood vessels. Based on the size of the dominant vessel involved, Wegener’s granulomatosis (WG) and the subgroups of polyarteritis nodosa (PAN), such as microscopic polyangiitis or polyarteritis (MPA) and Churg‐ Strauss syndrome (CSS) belong to the same group of necrotizing vasculitides aAecting mainly small-sized vessels. It should be noted that these vasculitides (WG, MPA and CSS) are associated with the |
| Databáze: | OpenAIRE |
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