Wegener's granulomatosis strictly and persistently localized to one organ is rare: assessment of 16 patients from the French Vasculitis Study Group database.
OBJECTIVE
To study the frequency and characteristics of patients with Wegener's granulomatosis (WG) strictly and persistently localized to one organ.


METHODS
Retrospective analysis of the French Vasculitis Study Group (FVSG) WG cohort.


RESULTS
Sixteen patients (3.2% of the cohort) were identified who had isolated lung nodules, ear-nose-throat, or ocular involvement that did not progress to systemic disease (median followup, 58 mo) over the period of observation. Ten received first-line therapy with cyclophosphamide, which was effective in 4. Cotrimoxazole alone achieved remission in one, combined with corticosteroids in 3. Eight required subsequent treatments because of first-line failure or relapse.


CONCLUSIONS
Strictly and persistently localized WG is uncommon. Optimal treatment remains to be determined.
Department of Internal Medicine, Hôpital Cochin, 75879 Paris Cedex 14, France. christian.pagnoux@cch.aphp.fr
Christian
C
Pagnoux
Muriel
M
Stubbe
François
F
Lifermann
Olivier
O
Decaux
Michel
M
Pavic
Alice
A
Bérezné
Isabelle
I
Delacroix-Szmania
Nadine
N
Méaux-Ruault
Boris
B
Bienvenu
Jean
J
Cabane
Loïc
L
Guillevin
D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D003520:Cyclophosphamide / Q000627:therapeutic use; D016208:Databases, Factual; D005260:Female; D005602:France; D014890:Granulomatosis with Polyangiitis / Q000188:drug therapy / Q000473:pathology* / Q000503:physiopathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D012008:Recurrence; D012074:Remission Induction; D012189:Retrospective Studies; D055815:Young Adult
D000305:Adrenal Cortex Hormones; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide