From: Biopsy-proven kidney involvement in hypocomplementemic urticarial vasculitis
Patients report | Litterature review | p | Total | |
---|---|---|---|---|
Number of patients | 12 | 26 | 38 | |
Age at diagnosis (years), mean | 42 | 32 | 0.14 | 35 |
Female gender, n (%) | 9 (75%) | 16 (61%) | 0.49 | 25 (66%) |
Clinical examination | ||||
Asthenia/fever | 10 (83%) | 13 (50%) | 0.07 | 23 (60%) |
Chronic urticaria | 12 (100%) | 26 (100%) | 1 | 38 (100%) |
Angioedema/purpura/livedo | 7 (58%) | 13 (50%) | 0.73 | 20 (53%) |
Cutaneous leucocytoclastic vasculitis | 7/9 (78%) | 18/18 (100%) | 0.1 | 25/27 (92%) |
Arthralgia/arthritis | 8 (67%) | 13 (50%) | 0.49 | 21 (55%) |
Anterior uveitis/conjunctivitis | 4 (33%) | 8 (30%) | 1 | 12 (32%) |
Retinopathy | 2 (17%) | 1 (3%) | 0.23 | 3 (8%) |
Lymphadenopathy | 5 (42%) | 5 (19%) | 0.23 | 10 (26%) |
PRESS/peripheral neuropathy | 2 (17%) | 2 (7%) | 0.58 | 4 (11%) |
Abdominal pain | 1 (8%) | 10 (38%) | 0.12 | 11 (29%) |
Respiratory disease | 3 (25%) | 5 (19%) | 0.69 | 8 (21%) |
Cardiac involvement | 1 (8%) | 3 (11%) | 1 | 4 (11%) |
Renal presentation | ||||
Proteinuria | 12 (100%) | 22 (84%) | 0.29 | 34 (89%) |
Nephrotic syndrom | 6 (50%) | 7 (26%) | 0.27 | 13 (34%) |
Microscopic/macroscopic hematuria | 9 (75%) | 17 (65%) | 0.71 | 26 (68%) |
Acute kidney injury | 8 (67%) | 10 (38%) | 0.16 | 18 (47%) |
Hemodialysis at diagnosis | 2 (17%) | 1 (3%) | 0.23 | 3 (8%) |
High blood pressure | 7 (58%) | 3 (11%) | 0.005 | 9 (24%) |
Biology | ||||
Elevated CRP, fibrinogen or ESR | 5 (42%) | 10 (38%) | 1 | 15 (39%) |
Low C3 | 10 (83%) | 23 (88%) | 0.64 | 32 (84%) |
Low C4 | 11 (92%) | 21 (80%) | 0.64 | 31 (82%) |
Low CH50 | 9 (75%) | 14 (53%) | 0.29 | 22 (58%) |
Low C1q | 7 (58%) | 10 (38%) | 0.31 | 17 (45%) |
AntiC1q antibody | 8 (67%) | 10 (38%) | 0.16 | 17 (45%) |
Antinuclear antibody | 7 (58%) | 8 (30%) | 0.16 | 14 (37%) |
Antiphospholipid antibody | 1 (8%) | 1 (3%) | 0.54 | 2 (5%) |
Rheumatoid factor | 2 (17%) | 2 (7%) | 0.58 | 4 (11%) |
Cryoglobulinemia | 5 (42%) | 0 | 0.002 | 3 (8%) |
ANCA | 6 (50%) | 3 (11%) | 0.02 | 8 (21%) |
Monoclonal gammopathy | 1 (8%) | 0 | 0.32 | 1 (3%) |
Renal biopsy | ||||
Membranoproliferative GN | 8 (67%) | 15 (57%) | 0.73 | 23 (61%) |
Membranous nephritis | 0 | 3 (11%) | 0.54 | 3 (8%) |
Lupus like GN (diffuse deposits only) | 0 | 5 (19%) | 0.16 | 5 (13%) |
Crescentic GN/vasculitis | 3 (25%) | 2 (7%) | 0.30 | 5 (13%) |
Interstitial nephritis | 1 (8%) | 1 (3%) | 0.54 | 2 (5%) |
Mean duration of follow up (months) | 107 | 44 | 0.002 | 71 |
Deceased | 0 | 5 (19%) | 0.16 | 5 (13%) |
End-stage kidney disease | 4 (33%) | 4 (15%) | 0.23 | 8 (21%) |
Transplantation/Dialysis | 1 (8%)/2 (17%) | 1 (3%)/3 (11%) | 0.54/0.54 | 2 (5%)/5 (13%) |
eGFR: 15-60 ml/min/1.73 m2 | 2 (17%) | 3 (11%) | 0.64 | 5 (13%) |
Persistant proteinuria (> 0.3 g/24 h or g/g) | 4 (33%) | 4 (15%) | 0.23 | 8 (21%) |