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Primary systemic vasculitis: clinical features and mortality

S E Lane1, R A Watts, L Shepstone

  • 1Department of Rheumatology, Ipswich Hospital, Heath Rd, Ipswich IP4 5PD, UK. suzanne.lane@ipswichhospital.nhs.uk

QJM : Monthly Journal of the Association of Physicians
|January 19, 2005
PubMed
Abstract

Insights

Microscopic polyangiitis (MPA) and other primary systemic vasculitides (PSV) were described from a general hospital. MPA showed distinct clinical features and a potentially poorer prognosis compared to Wegener's granulomatosis (WG) and Churg Strauss syndrome (CSS).

Area of Science:

  • Rheumatology
  • Immunology
  • Internal Medicine

Background:

  • Primary systemic vasculitides (PSV), including Wegener's granulomatosis (WG), Churg Strauss syndrome (CSS), and microscopic polyangiitis (MPA), are typically described from specialized tertiary centers.
  • Understanding the clinical spectrum of these conditions in a general hospital setting is crucial for broader diagnostic and management insights.

Purpose of the Study:

  • To present the first clinical description of microscopic polyangiitis (MPA) originating from a general hospital setting.
  • To compare the clinical characteristics of MPA with those of Wegener's granulomatosis (WG) and Churg Strauss syndrome (CSS).

Main Methods:

  • A retrospective analysis of 99 primary systemic vasculitis (PSV) patient records from a single general hospital between 1988 and 2000.
  • Data collected included clinical features, demographics, diagnostic criteria (ACR, CHCC, Lanham), anti-neutrophil cytoplasmic antibody (ANCA) status, treatment, comorbidities, mortality, Birmingham vasculitis activity scores (BVAS), and vasculitis damage index (VDI).
  • Survival analysis was performed using Cox proportional hazards models and standardized mortality ratios (SMRs).

Main Results:

  • Microscopic polyangiitis (MPA) cases exhibited increased ear, nose, throat (ENT), and respiratory involvement but reduced renal involvement compared to previous reports.
  • Churg Strauss syndrome (CSS) demonstrated high rates of neurological, cardiovascular, and gastrointestinal involvement, along with the highest median VDI.
  • Microscopic polyangiitis (MPA) presented with a significantly lower BVAS than Wegener's granulomatosis (WG), and overall survival rates were lower for MPA (45.1%) compared to WG (75.9%) and CSS (68.1%).

Conclusions:

  • The clinical presentation of primary systemic vasculitides (PSV) in a general hospital aligns with findings from tertiary centers, though classification criteria for MPA require refinement.
  • Churg Strauss syndrome (CSS) is associated with significant neurological involvement and permanent damage.
  • Microscopic polyangiitis (MPA) may carry a poorer prognosis than Wegener's granulomatosis (WG) or Churg Strauss syndrome (CSS).

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