Table 5 Characteristics of SLE patients with cryoglobulinemia according to CryoVas status
From: Cryoglobulinemia in systemic lupus erythematosus: a retrospective study of 213 patients
SLE CG+ with CryoVas (n = 21) | SLE CG+ without CryoVas (n = 121) | p value | |
|---|---|---|---|
Female sex, n (%) | 16 (76%) | 108 (89%) | 0.15 |
SLE and CG diagnosis | |||
Age at lupus diagnosis, (years), mean (±SD) | 28.0 ± 14.1 | 30.0 ± 12.0 | 0.54 |
Years between SLE diagnosis and first positive CG, mean (±SD) | 7.6 ± 9.1 | 5.9 ± 7.1 | <0.0001 |
Years between SLE and CryoVas diagnosis, mean (±SD) | 8.5 ± 9.7 | NA | |
SLEDAI score at SLE diagnosis, mean (±SD) | 17.0 ± 7.5 | 13.6 ± 7.4 | 0.03 |
SLEDAI score at CG diagnosis, mean (±SD) | 17.9 ± 9.0 | 14.3 ± 8.9 | 0.08 |
SLEDAI score at CryoVas diagnosis, mean (±SD) | 23.7 ± 9.9 | NA | |
Associated autoimmune disorder | |||
Antiphospholipid syndrome, n (%) | 7 (33%) | 30 (25%) | 0.42 |
Sjogren’s syndrome, n (%) | 4 (19%) | 24 (20%) | 1.00 |
SLE clinical manifestations | |||
Acute cutaneous lupus, n (%) | 13 (62%) | 56 (46%) | 0.19 |
Subacute cutaneous lupus, n (%) | 10 (48%) | 31 (26%) | 0.04 |
Chronic cutaneous lupus, n (%) | 5 (24%) | 21 (17%) | 0.54 |
Oral ulcerations, n (%) | 5 (24%) | 24 (20%) | 0.54 |
Alopecia, n (%) | 9 (43%) | 27 (22%) | 0.046 |
Raynaud’s phenomenon, n (%) | 15 (71%) | 55 (45%) | 0.03 |
Arthritis and/or arthralgia, n (%) | 21 (100%) | 111 (92%) | 0.36 |
Pericarditis, n (%) | 11 (52%) | 23 (19%) | <0.0001 |
Myocarditis, n (%) | 2 (10%) | 5 (4%) | 0.28 |
Pleuritis, n (%) | 2 (10%) | 17 (14%) | 0.74 |
Valvulopathy, n (%) | 3 (14%) | 6 (5%) | 0.13 |
Intra-alveolar hemorrhage, n (%) | 1 (5%) | 1 (1%) | 0.27 |
Pulmonary arterial hypertension, n (%) | 0 (0%) | 1 (1%) | 1.00 |
Nephritis, n (%) | 9 (43%) | 55 (45%) | 0.97 |
Peripheral nervous system, n (%) | 2 (10%) | 2 (2%) | 0.08 |
Central nervous system, n (%) | 3 (14%) | 14 (12%) | 0.72 |
Psychosis, n (%) | 5 (24%) | 22 (18%) | 0.55 |
Leukopenia, n (%) | 6 (29%) | 30 (25%) | 0.71 |
Lymphopenia, n (%) | 15 (71%) | 74 (61%) | 0.37 |
Thrombopenia, n (%) | 6 (29%) | 30 (25%) | 0.71 |
Autoimmune hemolytic anemia, n (%) | 3 (14%) | 20 (17%) | 1.00 |
Immunology | |||
RF in serum positive, n (%) | 1 (5%) | 5 (4%) | 1.00 |
RF in serum, UI/L, mean (±SD) | 25 ± 4.2 | 137 ± 100 | 0.02 |
RF in cryoglobulin positive, n (%) | 0 (0%) | 3 (2%) | 1.00 |
Hypogammaglobulinemia, n (%) | 0 (0%) | 6 (5%) | 0.59 |
Hypergammaglobulinemia, n (%) | 16 (76%) | 71 (59%) | 0.13 |
Gammaglobulins, g/L, mean (±SD) | 17.4 ± 4.0 | 15.9 ± 5.9 | 0.17 |
Decreased complement C3, n (%) | 17 (81%) | 79 (65%) | 0.16 |
Decreased complement C4, n (%) | 19 (90%) | 88 (73%) | 0.08 |
Decreased CH50, n (%) | 17 (81%) | 74 (61%) | 0.08 |
Farr test, UI/L, mean (±SD) | 86.4 ± 82.1 | 69.9 ± 86.3 | 0.48 |
Total concentration of cryoprecipitate, mg/L, mean (range) | 31.1 (8.6-81.8) | 41.2 (6.1-228) | 0.06 |
Type I cryoglobulinemia, n (%) | 0 (0%) | 1 (1%) | |
Type II cryoglobulinemia, n (%) | 4 (19%) | 23 (19%) | 1.00 |
Monoclonal IgMκ + polyclonal IgG, n (%) | 0 (0%) | 14 (12%) | 0.13 |
Type III cryoglobulinemia, n (%) | 17 (81%) | 97 (80%) | 1.00 |
Polyclonal IgG + polyclonal IgM, n (%) | 12 (57%) | 61 (50%) | 0.43 |
Polyclonal IgG, n (%) | 4 (19%) | 18 (15%) | 0.74 |
Vasculitis features | |||
Purpura, n (%) | 9 (43%) | 0 (0%) | <0.0001 |
Acrocyanosis, n (%) | 4 (19%) | 11 (9%) | 0.24 |
Digital ischemia, n (%) | 6 (29%) | 3 (2%) | 0.02 |
Cutaneous necrosis, n (%) | 8 (38%) | 1 (1%) | <0.0001 |
Ulcers, n (%) | 8 (38%) | 8 (7%) | 0.0004 |
Livedo, n (%) | 7 (33%) | 16 (13%) | 0.047 |
Other manifestations | |||
Arthritis and/or arthralgia, n (%) | 18(86%) | 69 (57%) | 0.01 |
Peripheral nervous system, n (%) | 2 (10%) | 1 (1%) | 0.06 |
Central nervous system, n (%) | 4 (19%) | 5 (4%) | 0.03 |
Proteinuria > 1g/24h, n (%) | 4 (19%) | 18 (15%) | 0.74 |
Proteinuria > 3 g/24h, n (%) | 2 (10%) | 14 (12%) | 1.00 |
Hematuria, n (%) | 3 (14%) | 30 (25%) | 0.41 |
Kidney failure, n (%) | 1 (5%) | 11 (9%) | 1.00 |
Gastro-intestinal involvement, n (%) | 1 (5%) | 0 (0%) | 0.15 |
IS and immunomodulatory treatments | |||
Number of IS treatments, median (range) | 2 (0-7) | 2 (0-6) | 0.50 |
Corticosteroids, n (%) | 20 (95%) | 117 (97%) | 0.56 |
Methotrexate, n (%) | 7 (33%) | 49 (40%) | 0.52 |
Azathioprine, n (%) | 9 (43%) | 35 (29%) | 0.20 |
Mycophenolate mofetil, n (%) | 11 (52%) | 50 (41%) | 0.35 |
Belimumab, n (%) | 2 (10%) | 26 (21%) | 0.25 |
Anti-TNF agents, n (%) | 1 (5%) | 2 (2%) | 0.38 |
Cyclophosphamide, n (%) | 7 (33%) | 39 (32%) | 0.92 |
Rituximab, n (%) | 8 (38%) | 16 (13%) | 0.01 |
Calcineurin inhibitors, n (%) | 2 (10%) | 6 (5%) | 0.34 |
Thalidomide, n (%) | 1 (5%) | 9 (7%) | 1.00 |
Lenalidomide, n (%) | 0 (0%) | 1 (1%) | 1.00 |
Plasmapheresis, n (%) | 0 (0%) | 5 (4%) | 1.00 |