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Cryoglobulinemic vasculitis
Last reviewed: 07.07.2025
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Cryoglobulinemic vasculitis is a vasculitis with cryoglobulinemic immune deposits affecting small vessels (capillaries, venules, arterioles) mainly of the skin and glomeruli of the kidneys and combined with serum cryoglobulinemia. Infection with the hepatitis C virus is considered as an etiologic factor of the disease.
[ How does cryoglobulinemic vasculitis manifest itself?
Cryoglobulinemic vasculitis occurs predominantly in women around 50 years of age. The clearest sign of the disease is palpable purpura localized on the lower extremities, less often in the abdomen and buttocks. Urticaria and livedo reticularis may also occur. RF-positive leukocytoclastic vasculitis. Symmetrical migrating polyarthralgia without morning stiffness is characteristic, affecting the proximal interphalangeal, metacarpophalangeal and knee joints, less often the ankle and elbow joints. Most patients have signs of polyneuropathy (paresthesia and numbness of the lower extremities). Raynaud's phenomenon occurs in a third of patients. Sometimes these changes are the first signs of the disease.
The detection rate of Sjogren's syndrome ranges from 14 to 40%. In the late stages of the disease, clinical signs of kidney damage occur. Microhematuria, proteinuria, nephrotic syndrome and arterial hypertension, liver damage are characteristic. Rarely, the course of the disease is complicated by abdominal pain, pulmonary hemorrhage and myocardial infarction.
Symptoms of lung damage in this disease are expressed insignificantly. Usually patients complain of dry cough, expiratory dyspnea during physical exertion, chest pain. Very rarely, lung damage such as diffuse alveolar hemorrhages and respiratory distress syndrome are encountered.
How to recognize cryoglobulinemic vasculitis?
The diagnostic sign of the disease is the presence of cryoglobulins in the blood serum. IgM RF is often found in high titers. It is assumed that the composition of cryoglobulins also includes the IgG1 subclass with RF activity, which is involved in vascular and kidney damage. As a rule, patients have reduced Clq, C4, C2 and CH50 covalent levels with normal C3 concentrations. It is believed that these changes reflect cold activation of complement. ANF is detected in more than half of the cases.
Chest radiographs show signs of interstitial fibrosis, pulmonary infiltrates, pleural thickening and, rarely, cavities. Functional tests indicate deterioration of the diffusion capacity of the lungs and pathology of the small bronchi. Morphological changes include inflammation of small and medium arteries. Bronchoalveolar lavage data obtained from patients without clinical signs of pulmonary pathology indicate euclinical T-lymphocyte alveolitis (decrease in the number of alveolar macrophages and increase in CD3 T-lymphocytes).
Treatment of cryoglobulinemic vasculitis
Cryoglobulinemic vasculitis is treated with glucocorticosteroids, plasmapheresis in combination with antiviral drugs, preferably ribavirin. If they are ineffective, cyclophosphamide is used. A good effect of rituximab has been noted.
The most common causes of death in this disease are liver and kidney damage, cardiovascular diseases and lymphoproliferative diseases.