Management for cryoglobulinaemic vasculitis with organ involvement

For a management approach common to most of the systemic vasculitides, including the principles of inducing and maintaining remission of disease, see Management overview for systemic vasculitides.

If a patient has severe organ- or life-threatening features of cryoglobulinaemic vasculitis, refer them for specialist management. Management depends on their disease severity and must involve management for both the clinical features of cryoglobulinaemia and the aetiology (if known). The most common aetiologies are malignancies (especially lymphoproliferative disorders), infections (especially hepatitis C) and inflammatory connective tissue diseases (especially systemic lupus erythematosus [SLE] and Sjögren syndrome). Detailed information on the management for some of these aetiologies can be found in the following topics:

• hepatitis C infection
• hepatitis B infection
• human immunodeficiency virus (HIV) infection
• Sjögren syndrome
• SLE.

Managing the specific aetiology usually controls the associated cryoglobulinaemia; however, immediate intensive immunomodulatory drug therapy may be required for cryoglobulinaemic vasculitis associated with organ-threatening disease.

Short-term high-dose systemic corticosteroids (eg pulse intravenous or high-dose oral corticosteroids) may be beneficial in organ-threatening disease.

Rituximab is a specific biological disease-modifying antirheumatic drug (bDMARD) that acts on the B-cell antigen CD20 inhibitor. Its action limits the proliferation of B-cells responsible for cryoglobulin productionGalli, 2019. It may be beneficial in severe cryoglobulinaemic vasculitis and can be continued long term in most patientsDe Vita, 2012. If indicated for the management of severe cryoglobulinaemic vasculitis, use:

If patients respond to the initial 2 doses of rituximab, treatment may be repeated (usually after at least 6 months) depending on disease activity. For more detail on considerations for use of rituximab, see Specific considerations for use of biological or targeted-synthetic disease-modifying antirheumatic drugs (b/tsDMARDs).

Some patients with life-threatening disease may require plasma exchange for removal of the cryoglobulinsDe Vita, 2012Galli, 2019.