Table 2.
Summary of study characteristics on risk factors for relapse in type 1 cryoglobulinemic vasculitis.
| Type 1 Cryoglobulinemic Vasculitis | ||||||||
|---|---|---|---|---|---|---|---|---|
| Study | Aetiology | Clinical manifestations | Immunological features | Intervention with follow-up duration (months) | Clinical response |
Immunological response | Treatment-associated adverse events | Outcome |
| Terrier (2013) (n = 64) |
MGUS 44% HM 56% |
Cutaneous 86% Articular 28% Neuropathy 44% Nephropathy 30% |
Median CG 1.55 g/L Median C3 0.89 Median C4 0.09 |
MGUS-related CV: CS ± PLEX/AKA/Chloraminophene/CYC/RTX/AZA HM-related CV: CS ± RTX based/AKA/Bortezomib/±Thalidomide Follow-up 46.2 months |
MGUS 35%, HM 19% in remission |
NR in NHL (LPL) |
13% of severe infections 13% RTX worsening vasculitis |
MGUS 39% relapse, 26% refractory to first-line therapy HM 48% relapse, 21% refractory to first-line therapy 1 RTX-associated CV flare; Death 7% |
| Néel (2014) (n = 36) |
NMMG 36% HM 64% (WM 52%, MM 17.39%, CLL 4.34%, NHL 23%) |
Cutaneous 75% Articular 19% Neuropathy 47% Nephropathy 31% (MPGN 75%) |
CG IgM 69.44% Mean CG 2.5 g/L IgG 30.55%. κ 72% |
HM—cytoreductive regimen NMMG—non-cytoreductive regimen ± PLEX Follow-up 63 months |
Not assessable for CV - heterogeneous therapeutics for underlying LPD |
Not described | Treatment AE and infections 27% | Older age; Renal involvement frequent with IgG CG; Death 25% |
| Sidana (2017) (n = 102) |
LPD 92% HCV 3.9% Unknown 3.9% |
Cutaneous 63% Articular 24% Neuropathy 32% Nephropathy 14% |
CG IgG 53% IgM 39% Polyclonal IgG/ IgM 5% Median cryocrit HM 10%; MGUS 5% |
CS/AKA/RTX alone or combination; novel myeloma drugs ± PLEX/ASCT Follow-up 50 months |
CR 66% PR 14% NR 21% |
PR in HM and MGUS (Cryocrit 8% and 1%) |
Not described | Neurological involvement impacts survival outcome |
| Lobbes (2018) (n = 4) |
MGUS 25% MGUS + pSS 25% MM 25% BCL 25% |
Cutaneous100% Articular 75% Neuropathy 25% |
CG 726–6,762 mg/L CG IgG-κ 14.7; IgG-λ (5.6 & 22.6); IgA-λ 13 g/L |
Bortezomib ± low dose CS | Noted in 75% | CR in cases 1,3 PR in cases 2,3 |
Pneumonia Worsening neuropathic pain |
Relapse 100% between 4 and 18 months; Death 25% with uncontrolled CV, 50% due to underlying disease |
AE, adverse event; AKA, alkylating agents; ASCT, autologous stem cell transplant; AZA, azathioprine; BCL, B-cell lymphoma; CR, complete response; CS, corticosteroids; CG, cryoglobulins; CLL, chronic lymphocytic leukaemia; CV, cryoglobulinemia vasculitis; CYC, cyclophosphamide; HCV, hepatitis C virus; HM, hematological malignancy; Ig, immunoglobulins; LPD, lymphoproliferative disorders; LPL, lymphoplasmacytic lymphoma; MGUS, monoclonal gammopathy of undetermined significance; MM, multiple myeloma; MPGN, membranoproliferative glomerulonephritis; NHL, non-Hodgkin’s lymphoma; NMMG, non-malignant monoclonal gammopathy; NR, no responders; PLEX, plasmapheresis; PR, partial response; RTX, rituximab; pSS, primary Sjögrens syndrome; PR, partial response; RTX, rituximab; WM, Waldenström macroglobulinemia.