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2013 ; 92
(5
): 273-284
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English Wikipedia
Life-Threatening Cryoglobulinemic Patients With Hepatitis C: Clinical Description
and Outcome of 279 Patients
#MMPMID23974248
Retamozo S
; Díaz-Lagares C
; Bosch X
; Bové A
; Brito-Zerón P
; Gómez ME
; Yagüe J
; Forns X
; Cid MC
; Ramos-Casals M
Medicine (Baltimore)
2013[Sep]; 92
(5
): 273-284
PMID23974248
show ga
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.
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