title: Primary systemic vasculitis: clinical features and mortality.
abstract: BACKGROUND
  Wegener's granulomatosis (WG), Churg Strauss syndrome (CSS) and microscopic polyangiitis (MPA) are primary systemic vasculitides (PSV), the clinical features of which have been described from tertiary centres.
  
  
  OBJECTIVE
  To provide the first clinical description of MPA from a general hospital and compare clinical features with WG and CSS.
  
  
  METHODS
  Retrospective analysis of patient records.
  
  
  METHODS
  Records of 99 PSV patients attending a single hospital, from 1988 to 2000, were reviewed for: clinical features, date/age at diagnosis, sex, duration of illness, anti-neutrophil cytoplasmic antibodies (ANCA), treatment, comorbidity and deaths. Cases were classified using ACR, CHCC and Lanham criteria/definitions. Birmingham vasculitis activity scores (BVAS) and damage index (VDI) were calculated. Survival was assessed using Cox proportional hazards model and standardized mortality ratios (SMRs).
  
  
  RESULTS
  Compared to previous reports there was more ENT (29%) and respiratory (29%) but less renal (92%) involvement in MPA, and less ENT involvement in WG (81%). CSS showed high neurological (72%), cardiovascular (28%) and gastrointestinal (17%) involvement and the highest median (range) VDI (p = 0.01 vs. WG; p = 0.001 vs. MPA). BVAS1 was significantly lower in MPA than in WG [median (range) 15 (4-29) vs. 21 (6-39), (p = 0.001)] but not in CSS [20 (7-28), p = 0.08]. SMR (95%CI) for PSV was 4.8 (3.0-6.6); 5-year survival was 45.1% for MPA, 75.9% for WG and 68.1% for CSS. Age was a significant risk, but only to the same extent as in the reference population. When age was adjusted for, no other significant factor was found.
  
  
  CONCLUSIONS
  The clinical characteristics seen here are similar to those in previous series. There are difficulties in using the MPA CHCC definitions in classification. There is a high proportion of neurological involvement in CSS, causing permanent damage. MPA may have a poorer prognosis than WG or CSS.
authors.affiliation: Department of Rheumatology, Ipswich Hospital, Heath Rd, Ipswich IP4 5PD, UK. suzanne.lane@ipswichhospital.nhs.uk
authors.forename: S E
authors.initials: SE
authors.lastname: Lane
authors.forename: R A
authors.initials: RA
authors.lastname: Watts
authors.forename: L
authors.initials: L
authors.lastname: Shepstone
authors.forename: D G I
authors.initials: DG
authors.lastname: Scott
mesh_terms: D000368:Aged; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000032:analysis; D015267:Churg-Strauss Syndrome / Q000145:classification / Q000401:mortality / Q000473:pathology; D003520:Cyclophosphamide; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000145:classification / Q000401:mortality / Q000473:pathology; D006801:Humans; D007674:Kidney Diseases / Q000145:classification / Q000401:mortality / Q000473:pathology; D008297:Male; D008875:Middle Aged; D010038:Otorhinolaryngologic Diseases / Q000145:classification / Q000401:mortality / Q000473:pathology; D010488:Polyarteritis Nodosa / Q000145:classification / Q000401:mortality / Q000473:pathology; D012140:Respiratory Tract Diseases / Q000145:classification / Q000401:mortality / Q000473:pathology; D012189:Retrospective Studies; D014657:Vasculitis / Q000145:classification / Q000401:mortality / Q000473:pathology*
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D003520:Cyclophosphamide