Cryoglobulininemia는 환자의 혈청에 cryoglobulin이 존재하는 것이고 이것이 small-to medium-sized blood vessels에 영향을 미쳐 증상을 나타낸 것이 cryoglobulinemic vasculitis입니다.
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●Cryoglobulin – Cryoglobulins are Igs in the serum that precipitate at temperatures below 37°C and redissolve on rewarming. The appearance of precipitated cryoglobulin is illustrated in the picture (picture). ●Cryoglobulinemia – Cryoglobulinemia refers to the presence of cryoglobulin in a patient's serum. This term is sometimes used interchangeably with cryoglobulinemic vasculitis (or cryoglobulinemia syndrome). ●Cryoglobulinemic vasculitis – Cryoglobulinemic vasculitis (or cryoglobulinemia syndrome) refers to a systemic inflammatory syndrome that generally involves small-to-medium vessel vasculitis due to cryoglobulin-containing immune complexes. This term is generally used to distinguish the asymptomatic presence of cryoglobulins from the clinically apparent disorder with end-organ damage caused by cryoglobulins precipitating in small- to medium-sized blood vessels. |
Serum cryoglobulin qualitative test
(A) Serum cryoprecipitates appeared after 7 days of incubation at 4°C.
(B) Serum cryoprecipitates redissolved after rewarming at 37°C.
임상적으로 의미 있는 cryoglobulinemia의 유병률은 약 10만명 당 1명이지만 임상 증상이 없이 circulating cryoglobulins만 검출되는 환자들은 만성 감염 또는 염증 환자에서 의미 있게 높습니다. HIV 환자들에서 15-20 %, 결체조직질환에서 15-25 %, 만성C형간염에서 40-65 %, HIV와 만성C형간염이 같이 있는 경우는 64 %까지 높습니다.
어느 때 진단을 의심해야 하는가?
혈액질환, 바이러스 감염, 결체조직질환 환자에서 관절통, 자반, 피부 궤양, 말초 신경병증이 있는 경우에 의심을 크게 합니다.
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The range of clinical presentations associated with the various cryoglobulinemia syndromes is broad, but some of the more common features include arthralgia, purpura, skin ulcers, glomerulonephritis, and peripheral neuropathy. The index of suspicion for cryoglobulinemia should be raised further if these occur in the setting of a clonal hematologic disease (eg, multiple myeloma, Waldenström macroglobulinemia), viral infection (eg, hepatitis C virus [HCV], hepatitis B virus [HBV]), or connective tissue disease (eg, systemic lupus erythematosus [SLE], Sjögren's syndrome, or rheumatoid arthritis). Overall, the presence of a measurable amount of cryoglobulin (cryocrit) with a low C4 complement level remain the most prominent laboratory hallmarks of cryoglobulinemia syndromes. Several other serologic findings have often been reported, but none are pathognomonic since they are often encountered in healthy individuals and/or other confounding medical conditions. |
필요한 혈액검사는 다음과 같습니다. Cryoglobulinemia와 관련하여 시행할 바이러스 검사는 HBV, HCV, HIV입니다.
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The presence of a measurable amount of cryoglobulin (cryocrit) with a low C4 complement level remain the most prominent laboratory hallmarks of cryoglobulinemia syndromes. The diagnostic evaluation for a case of possible cryoglobulinemic vasculitis should include a detailed history including infectious disease exposure (eg, hepatitis B or C virus, HIV), a prior history of a clonal hematologic disease (eg, multiple myeloma, Waldenström macroglobulinemia, MGUS) or systemic autoimmune disease (especially SLE or Sjögren's syndrome), and symptoms or manifestations that may characterize or exclude a suspected diagnosis; a careful physical examination; laboratory testing to include detection of cryoglobulins, serum chemistries, urinalysis, complement (especially C4 level), RF, viral serologies (particularly for HBV, HCV, and HIV), acute phase reactants, as well as other autoantibodies such as antinuclear antibodies (ANAs) and antineutrophil cytoplasmic antibody (ANCA). Serologic studies, particularly for viral hepatitis, are always indicated during the laboratory evaluation of a patient with mixed cryoglobulinemia. Hepatitis C has been reported in 60 to 90 percent of patients with mixed cryoglobulinemia syndromes, but other populations may have lower rates. Serologic studies for other agents, including hepatitis B, HIV, and Epstein-Barr virus, have been somewhat inconsistent but are generally worth at least considering during the workup of cryoglobulinemia |
REF. UpToDate 2020.08.19
