Life-Threatening Cryoglobulinemic Patients With Hepatitis C

Abstract

Cryoglobulinemia is characterized by a wide range of causes, symptoms, and outcomes. Hepatitis C virus (HCV) infection is detected in 30%–100% of patients with cryoglobulins. Although more than half the patients with cryoglobulinemic vasculitis present a relatively benign clinical course, some may present with potentially life-threatening situations. We conducted the current study to analyze the clinical characteristics and outcomes of HCV patients presenting with life-threatening cryoglobulinemic vasculitis. We evaluated 181 admissions from 89 HCV patients diagnosed with cryoglobulinemic vasculitis consecutively admitted to our department between 1995 and 2010. In addition, we performed a systematic analysis of cases reported to date through a MEDLINE search.The following organ involvements were considered to be potentially life-threatening in HCV patients with cryoglobulinemic vasculitis: cryoglobulinemic, biopsy-proven glomerulonephritis presenting with renal failure; gastrointestinal vasculitis; pulmonary hemorrhage; central nervous system (CNS) involvement; and myocardial involvement. A total of 279 patients (30 from our department and 249 from the literature search) fulfilled the inclusion criteria: 205 presented with renal failure, 45 with gastrointestinal vasculitis, 38 with CNS involvement, 18 with pulmonary hemorrhage, and 3 with myocardial involvement; 30 patients presented with more than 1 life-threatening cryoglobulinemic manifestation. There were 146 (52%) women and 133 (48%) men, with a mean age at diagnosis of cryoglobulinemia of 54 years (range, 25–87 yr) and a mean age at life-threatening involvement of 55 years (range, 25–87 yr). In 232 (83%) patients, life-threatening involvement was the first clinical manifestation of cryoglobulinemia. Severe involvement appeared a mean of 1.2 years (range, 1–11 yr) after the diagnosis of cryoglobulinemic vasculitis. Patients were followed for a mean of 14 months (range, 3–120 mo) after the diagnosis of life-threatening cryoglobulinemia. Sixty-three patients (22%) died. The main cause of death was sepsis (42%) in patients with glomerulonephritis, and cryoglobulinemic vasculitis itself in patients with gastrointestinal, pulmonary, and CNS involvement (60%, 57%, and 62%, respectively). In conclusion, HCV-related cryoglobulinemia may result in progressive (renal involvement) or acute (pulmonary hemorrhage, gastrointestinal ischemia, CNS involvement) life-threatening organ damage. The mortality rate of these manifestations ranges between 20% and 80%. Unfortunately, this may be the first cryoglobulinemic involvement in almost two-thirds of cases, highlighting the complex management and very elevated mortality of these cases.

Extrahepatic manifestations of chronic hepatitis C virus infection

“…In a retrospective Italian study of 231 patients, 79/97 deaths were linked to vasculitis (46%, of which one-third were due to renal involvement), cancer or hemopathy (23%), or liver disease (13%) [Ferri et al 2004]. Cryovas complications may result in progressive (renal involvement) or acute (pulmonary hemorrhage, gastrointestinal ischemia, cardiac, central nervous system involvement) life-threatening organ damage, with a mortality rate between 20% and 80% [Retamozo et al 2013;Terrier et al 2013]. Intestinal ischemia, pulmonary hemorrhage, high cryocrit levels and type II MC are associated with severe prognosis [Ramos-Casals et al 2006].…”

HCV Treatments and Their Integration Into Rheumatology

“…In approximately one third of the patients, the disease follows an aggressive course [21] while in 15 % life-threatening complications can occur [24]. Overall, the 10-year survival rate ranges between 56 and 80 % [21][22][23]; baseline factors associated with a poor prognosis include advanced liver fibrosis, male sex, and advanced age [21,22].…”

Dermatolojide Rituksimab Kullanımı

“…Benzer tip 2 kriyoglobulinemili hasta da rituksimaba yanıt verebilirken, başka bir hasta ise rituksimab ve infliksimab kombinasyonuna yanıt vermiştir. Bu olgu bildirilerindeki iyi yanıt varlığına karşın, esansiyel mikst kriyoglobulinemilerde genellikle rituksimaba yanıt alınamamak-tadır (124)(125)(126).…”