Table 1 Clinical and biological presentation of the 12 patients from the present cohort
From: Biopsy-proven kidney involvement in hypocomplementemic urticarial vasculitis
Patient 1 | Patient 2 | Patient 3 | Patient 4 | Patient 5 | Patient 6 | Patient 7 | Patient 8 | Patient 9 | Patient 10 | Patient 11 | Patient 12 | |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
Age at kidney involvement | 42 | 12 | 41 | 40 | 41 | 5 | 44 | 56 | 55 | 76 | 45 | 43 |
Gender | F | F | F | F | F | M | F | F | F | M | F | F |
Extra-renal involvement | ||||||||||||
Asthenia/fever | +/+ | +/+ | +/− | +/− | +/− | −/+ | +/− | −/− | −/− | +/+ | +/+ | +/− |
Chronic urticaria | + | + | + | + | + | + | + | + | + | + | + | + |
Angioedema/purpura/livedo | −/+/+ | −/−/− | −/+/− | +/−/− | +/−/− | −/−/− | +/−/− | −/−/− | +/−/− | −/+/− | −/−/− | −/−/− |
Cutaneous leucocytoclastic vasculitis | + | – | + | + | + | ND | – | + | ND | + | ND | + |
Arthralgia/arthritis | +/+ | +/+ | +/− | −/− | +/+ | −/− | +/− | −/− | +/− | +/− | −/− | +/− |
Anterior uveitis/conjunctivitis | +/+ | −/+ | −/− | −/− | +/− | −/− | −/− | −/− | −/− | −/− | −/− | +/− |
Retinopathy | + | – | – | – | – | – | – | + | – | – | – | – |
Lymphadenopathy | + | – | – | – | + | – | + | – | + | – | – | + |
PRESS/peripheral neuropathy | +/− | +/− | −/− | −/− | −/− | −/− | −/− | −/− | −/− | −/− | −/− | −/− |
Respiratory disease | – | + | – | + | – | – | – | + | – | – | – | – |
Pericarditis | + | – | – | – | – | – | – | – | – | – | – | – |
Renal presentation | ||||||||||||
Time since first symptoms of HUV (months) | 84 | 22 | 37 | 55 | 49 | 42 | 60 | 0,5 | 36 | 76 | 0 | 6 |
Proteinuria | + | + | + | + | + | + | + | + | + | + | + | + |
Nephrotic syndrome | + | – | + | + | – | + | + | – | + | – | – | – |
Microscopic/macroscopic haematuria | +/− | −/− | +/+ | +/+ | −/− | +/+ | +/+ | +/− | +/+ | +/− | −/− | +/− |
Serum creatinine (μmol/L) | 225 | 703 | 325 | 90 | 71 | ND | 160 | 77 | 326 | 608 | 268 | 140 |
AKI | + | + | + | – | – | – | + | – | + | + | + | + |
Hemodialysis at diagnosis | – | + | – | – | – | – | – | – | – | + | – | – |
High blood pressure | + | + | – | – | – | – | + | + | + | + | – | + |
Pathological diagnosis | MPGN | MPGN | MPGN | MPGN | MPGN | MPGN | MPGN | MPGN | PI CGN | PI CGN | NV | IN |
Biology | ||||||||||||
Elevated CRP | + | ND | ND | ND | ND | ND | ND | – | + | + | + | + |
Low C3 | + | + | + | + | + | + | + | + | ND | + | ND | + |
Low C4 | + | + | + | + | + | + | + | + | + | + | ND | + |
Low CH50 | + | + | + | + | + | + | ND | + | ND | + | ND | + |
Low C1q | + | + | + | ND | + | ND | ND | ND | + | + | ND | + |
Anti-C1q antibody | + | ND | + | – | + | ND | + | + | + | – | + | + |
Antinuclear antibody | – | + | – | + | – | – | + | + | + | – | + | + |
Antiphospholipid antibody | – | – | ND | – | ND | ND | ND | ND | ND | – | + | ND |
Rheumatoid factor | + | – | – | – | – | – | – | ND | ND | – | – | + |
Cryoglobulinemia | + | – | – | – | – | – | + | + | ND | + | – | + |
ANCA (anti-MPO/anti-PR3) | – | + (+/−) | – | – | – | – | + (+/−) | + (−/+) | + (+/−) | – | + (−/−) | + (−/−) |
Monoclonal gammapathy | – | – | – | – | – | – | – | – | – | + | – | – |