Clinical features and outcomes of 37 Argentinean patients with severe granulomatosis with polyangiitis (wegener granulomatosis).
BACKGROUND
Most epidemiologic studies involving severe granulomatosis with polyangiitis (SGPA) patients have investigated populations from the northern hemisphere, whereas few studies have been conducted in South America. None of the South American studies have differentiated between localized GPA and SGPA.


OBJECTIVE
The present study was designed to describe a cohort of Argentinean patients who were diagnosed with SGPA and to compare this cohort with previously well-described cohorts.


METHODS
We performed a retrospective study that included 37 consecutive SGPA patients who were seen at 2 tertiary centers in Buenos Aires.


RESULTS
Nineteen patients (51.3%) were male, and 18 patients (49.7%) were female. The mean age at the onset of symptoms was 48.5 ± 12.01 years. Antineutrophil cytoplasmic antibody (ANCA) was detected in 34 patients (91.89%): 32 patients (86.48%) had a cytoplasmic staining pattern, whereas 2 patients (5.40%) had a perinuclear pattern. Three patients were ANCA-negative. Twenty-four patients (64%) achieved remission, and 7 patients (19%) had response as defined by at least 50% reduction in the disease activity score. Nineteen relapses were observed in 12 patients, and 2 of the relapses were fatal. Overall, there were 14 deaths (37.83%).


CONCLUSIONS
The present series demonstrated that Argentinean patients have similar demographics, clinical manifestations, and outcomes as the cohorts from the northern hemisphere. There was less granulomatous organ involvement (ear/nose/throat, lung granulomas) in the present cohort compared with other series.
Department of Rheumatology, Hospital Aeronáutico Central, Ciudad Autónoma de Buenos Aires, Argentina. aoorden@yahoo.com.ar
Alberto Omar
AO
Orden
Sebastián Andrés
SA
Muñoz
María Cristina
MC
Basta
Alberto
A
Allievi
D000328:Adult; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood; D001118:Argentina; D015331:Cohort Studies; D003520:Cyclophosphamide / Q000627:therapeutic use*; D004359:Drug Therapy, Combination; D005260:Female; D005455:Fluorescent Antibody Technique; D005938:Glucocorticoids / Q000627:therapeutic use*; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis* / Q000188:drug therapy* / Q000401:mortality; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D012074:Remission Induction; D012189:Retrospective Studies; D016896:Treatment Outcome
D019268:Antibodies, Antineutrophil Cytoplasmic; D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide