Transcript Hypocomplementemic urticarial vasculitis syndrome (HUVS) in kidney biopsies (PPTX / 18958.44 KB) - big file, download may take a while

Hypocomplementemic urticarial
vasculitis syndrome (HUVS)
in kidney biopsies
Alenka Vizjak, Institute of Pathology, Faculty of Medicine, Ljubljana
Jerica Mraz, Institute of Pathology, Faculty of Medicine, Ljubljana
Tadej Avčin, University Children Hospital, Ljubljana
Alojzija Hočevar, Department of Rheumatology, University Medical
Centre, Ljubljana
Jelka Lindič, Department of Nephrology, University Medical Centre,
Ljubljana
Dušan Ferluga, Institute of Pathology, Faculty of Medicine, Ljubljana,
SLOVENIA
HUVS
• McDuffie et al (1973)
• Schwartz et al (1982) – criteria for HUVS:
Major: chronic recurrent urticaria
hypocomplementemia (↓ C1q, C4, C3)
Minor: leukocytoclastic vasculitis, arthralgia/
arthritis, glomerulonephritis, uveitis or episcleritis,
abdominal pain, anti-C1q antibodies
(2 major and 2 minor criteria must be fulfilled).
Exclusion criteria: ANA (high values), anti-DNA,
cryoglobulinemia, hepatitis B infection, hereditary
deficiency of complement
• Other clinical features: angioedema, obstructive
pulmonary disease, pulmonary hemorrhage,
pleural effusion, pericarditis, neurologic affection
HUVS
• Rare, autoimmune systemic disease,
ocurring mostly in the age of 30 – 40 years,
more frequently in women (2:1)
• Rare children with HUVS have been
described (among them 3 siblings, in our
study 2 siblings)
• Renal involvement affects up to 50% of
patients with HUVS (mild mesangial GN, focal
or diffuse mesangial proliferative GN,
mesangiocapillary GN, rarely membranous
and crescentic GN)
Anti-C1q antibodies
•
•
•
•
Directed against collagen-like region of C1q
Their pathogenetic role still not clarified
HUVS – positive in nearly 100%
Autoimmune systemic diseases, particularly
SLE – positive in 30-40%, in active lupus
nephritis – positive in about 80%
• Different forms of GN, particularly
mesangiocapillary GN – positive in 1%-50%
• Infections (HVC, HBV, HIV)
• Healthy subjects – positive in 0-3% in children
and up to 18% in older people
Table 1. Clinical and laboratory findings in
8 HUVS patients
Table 2. Kidney biopsy findings in 8 HUVS
patients with 14 kidney biopsies
Conclusions
• We confirmed immune-complex pathogenesis of
HUVS and in contrast to other studies regularly
found extensive extraglomerular tubulointerstitial
and vascular ꞌfull houseꞌ immune deposits as well
as occasional fingerprint deposits in kidneys
• Controversial opinion whether HUVS represent a
disease entity
• Majority of authors believe that HUVS could be
considered as a spectrum of manifestations that
may progress to SLE (about 50% of HUVS patients
develop SLE), or as a specific form of SLE
• It is also possible that HUVS might be primary or
secondary in association with an underlying
autoimmune systemic diseases, particularly SLE.