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Necrotizing vasculitis in Greece: clinical, immunological and immunogenetic aspects. A study of 66 patients
K A Boki1, U Dafni, G A Karpouzas
1Department of Pathophysiology, School of Medicine, National University of Athens, Greece.
Insights
This study compared Wegener
Area of Science:
- Rheumatology
- Immunology
- Internal Medicine
Background:
- Necrotizing vasculitis encompasses several distinct conditions with varying clinical presentations and outcomes.
- Understanding the differences and similarities in clinical expression, treatment response, and immunogenetics is crucial for effective patient management.
Purpose of the Study:
- To evaluate the clinical spectrum and outcomes of necrotizing vasculitis.
- To assess the response to various therapeutic approaches based on major disease events.
- To investigate the immunogenetic background of patients diagnosed with different types of vasculitis.
Main Methods:
- Retrospective study of 66 Greek patients diagnosed with vasculitis according to ACR criteria.
- Classification into Wegener's granulomatosis (WG), polyarteritis nodosa (PAN), and Churg-Strauss syndrome (CSS).
- Analysis of clinical manifestations, demographic characteristics, serological markers (c-ANCA, HBsAg), HLA antigens, and treatment responses.
Main Results:
- WG and PAN patients had similar demographics; PAN more frequently involved skin, GI tract, and peripheral nerves, while WG affected lungs, upper respiratory tract, kidneys, and ears.
- Muscle weakness was exclusive to PAN, asthma to CSS. c-ANCA was characteristic of WG, and HBsAg was found in 22% of PAN patients.
- No significant difference in the first major event between PAN and WG. Cyclophosphamide route impacted WG outcomes (P=0.006). WG showed increased DR1 frequency, PAN/CSS lacked DR3.
Conclusions:
- Despite differing immunogenetic backgrounds and clinical expressions, patients with WG and PAN exhibit similar treatment responses, disease evolution, and survival rates.
- Specific clinical features and serological markers aid in differentiating WG, PAN, and CSS.
- The route of cyclophosphamide administration may influence outcomes in WG patients.
Abstract:
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 (c-ANCA) characterized WG patients. Hepatitis B surface antigen (HBsAg) was found in 22% of PAN patients. No significant differences 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 difference was found on different 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 differ, the response to treatment as well as the evolution and the survival rate of these patients are similar in the two groups.