Cryoglobulinemic vasculitis
Last reviewed: 23.04.2024
All iLive content is medically reviewed or fact checked to ensure as much factual accuracy as possible.
We have strict sourcing guidelines and only link to reputable media sites, academic research institutions and, whenever possible, medically peer reviewed studies. Note that the numbers in parentheses ([1], [2], etc.) are clickable links to these studies.
If you feel that any of our content is inaccurate, out-of-date, or otherwise questionable, please select it and press Ctrl + Enter.
Cryoglobulinemic vasculitis - 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 hepatitis C virus is considered as an etiologic factor of the disease.
[How is cryoglobulinemic vasculitis manifested?
Cryoglobulinemia vasculitis predominantly occurs in women aged around 50 years. The purest sign of the disease is palpable purpura, localized on the lower extremities, less often in the abdomen and buttocks. It is also possible the emergence of urticaria, a reticulum. RF-positive leukocytoclastic vasculitis. Characteristic of the development of symmetrical migrating polyarthralgias without morning stiffness, affecting proximal interphalangeal, metacarpophalangeal and knee, less often - ankle and elbow joints. In most patients, signs of polyneuropathy (paresthesia and numbness of the lower extremities) are noted. A third of patients experience the phenomenon of Raynaud. Sometimes these changes are the first signs of the disease.
The frequency of detection of Sjogren's syndrome varies from 14 to 40%. In the late stages of the disease there are clinical signs of kidney damage. Typical microhematuria, proteinuria, nephrotic syndrome and hypertension, liver damage. Rarely, the course of the disease is complicated by abdominal pain, pulmonary hemorrhage and myocardial infarction.
Symptoms of lung damage in this disease are not very pronounced. Usually patients complain of dry cough, expiratory dyspnoea with physical exertion, chest pain. Extremely rare lesions of the lungs by the type of diffuse alveolar hemorrhages and respiratory distress syndrome.
How to recognize cryoglobulinemic vasculitis?
The diagnostic sign of the disease is the presence of cryoglobulins in the blood serum. Often found IgM RF in high titre. It is assumed that the composition of cryoglobulins also includes an IgG1 subclass with RF activity that takes part in the defeat of blood vessels and kidneys. As a rule, in patients reduced by a copy of the grace of Clq, C4, C2 and CH50 at normal C3 concentration. It is believed that these changes reflect the cold activation of complement. In more than half the cases ANF are found.
On radiographs of the lungs, signs of interstitial fibrosis, pulmonary infiltrates, thickening of the pleura and. Rarely, cavity. Functional tests indicate a deterioration in the diffusivity of the lungs and the 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 eubclinal T-lymphocyte alveolitis (a decrease in the number of alveolar macrophages and an increase in CD3 T-lymphocytes).
Treatment of cryoglobulinemic vasculitis
Cryoglobulinemic vasculitis is treated based on the use of glucocorticosteroids, plasmapheresis in combination with antiviral drugs, preferably ribavirin. When they are ineffective, cyclophosphamide is used. A good effect of rituximab was noted.
The most frequent causes of death in this disease are liver and kidney damage, diseases of the cardiovascular system and lymphoproliferative diseases.