P. Cacoub et al., SYSTEMIC VASCULITIS COMPLICATING INFECTIO NS - RECOVERY WITHOUT CORTICOTHERAPY, Journal des maladies vasculaires, 22(1), 1997, pp. 29-34
In the most cases the causes of systemic vasculitis are unknown and tr
eatment is symptomatic (corticosteroids often associated with immunosu
pressive agents). We report three cases of systemic vasculitis associa
ted with infections for which dramatic improvement was observed withou
t corticotherapy (in two patients). Case report 1 : A previously overw
eight 72-year-old woman was admitted because of a one-year history of
fever, fourteen kilogram weight loss, vascular purpura, and polyneurop
athy. Abnormal laboratory values included inflammatory syndrome [eryth
rocyte sedimentation rate (ESR) : 80 mm/first hour, thrombocytosis : 5
00 000/mu l, hypereosinophilia (1 200/mu l) and positive perinuclear a
nti-neutrophil cytoplasmic antibodies (p-ANCA) [anti-myeloperoxydase a
ntibodies : 30 U (normal < 7)]. Neuromuscular biopsy showed necrotizin
g vasculitis involving small and medium arteries. Further studies reve
aled a florid diverticulosis of the colon and no other severe visceral
involvement. Treatment was starred then with sigmoidectomy. Within si
x weeks her general condition improved dramatically without corticothe
rapy : regression of all systemic manifestations, the ESR normalized,
and p-ANCA became negative, Ten months later she was still asymptomati
c. Case report 2 : An 50-year-old-man developed a progressive fifteen-
kilogram weight loss (within 2 months). severe polyneuropathy of all f
our limbs, His ESR was 120 mm/first hour, and C-reactive protein 200 m
g/l. Neuromuscular biopsy showed necrotizing vasculitis affecting smal
l vessels in the nerve and no immune deposits. Stomatologic examinatio
n revealed a multiple foci of dental infections. The extraction of all
these foci of infections associated with antibiotics improved dramati
cally all systemic manifestations (within eight weeks), once more with
out corticotherapy. Light months later the patient remained asymptomat
ic. Case report 3 : A 30-year-old-woman was admitted because of five-w
eek history of fever, myalgias, polyarthritis, and cutaneous nodules i
n her limbs. Abnormal laboratory values included inflammatory syndrome
, proteinuria of 0,7 g/day, and a significant rise in Chlamydia tracho
matis antibodies titres from 1/64 to 1/256 over a 5 week period. She h
ad a previous history of genital condyloma. The prednisone initialed (
0,5 mg/kg/day) twelve days prior to admission was gradually reduced (s
topped within 2 months) and treatment with doxycycline (200 mg/day) wa
s initiated. Within six weeks of antibacterial treatment we assisted t
o a total regression of the initial clinical manifestations and labora
tory values became normalized. Ten months later she remained asymptoma
tic. Conclusion: In systemic vasculitis, investigations in a search of
foci of infections are of dual interest : possible etiologic agent li
ke our case reports (strong evidence for an infectious association) an
d, from a therapeutic perspective, we must identify the microbes behin
d vasculitis syndromes, since treatment with corticosteroids may have
serious consequences if the patient has an active infectious disease (
J Mal Vasc 1997 ; 22 : pages 29-34).