Life-threatening cryoglobulinemia: clinical and immunological characterization of 29 cases.
OBJECTIVE
To analyze the etiology, clinical presentation, and outcomes of patients with life-threatening cryoglobulinemic vasculitis.


METHODS
We studied 209 consecutive patients with cryoglobulinemic vasculitis. A potentially life-threatening cryoglobulinemia was considered as the development of renal failure, vasculitic abdominal involvement, pulmonary hemorrhage, or central nervous system involvement.


RESULTS
Twenty-nine (14%) patients had life-threatening cryoglobulinemic vasculitis. There were 17 women and 12 men, with a mean age of 57 years. In 17 (59%) patients, life-threatening cryoglobulinemia was the initial clinical feature of the disease. The 29 patients had a total of 33 life-threatening episodes, which included renal failure due to cryoglobulinemic glomerulonephritis (n = 18), intestinal vasculitis (n = 8), pulmonary hemorrhage (n = 4), and central nervous system involvement (n = 3). In comparison with a control group of age-sex-matched patients with milder cryoglobulinemic vasculitis, those with severe cryoglobulinemic vasculitis had a higher frequency of fever (28% versus 7%, P = 0.017), type II cryoglobulins (100% versus 59%, P = 0.008), low C3 levels (55% versus 20%, P = 0.001), and a higher mean value of cryocrit (11.4% versus 3.3%, P = 0.004). Nineteen (66%) of the 29 patients with life-threatening involvement died, with the mortality rate reaching 100% in patients with intestinal ischemia and pulmonary hemorrhage.


CONCLUSIONS
Life-threatening cryoglobulinemic vasculitis was observed in 14% of our patients, with almost two-thirds of episodes occurring at the onset of the disease. Fever, high cryocrit levels, and low C3 levels were associated with this severe presentation. Two-thirds of the patients died, with mortality for pulmonary hemorrhage and intestinal ischemia reaching 100%.
Ramos-Casals
Manuel
M
Department of Autoimmune Diseases, School of Medicine, University of Barcelona, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Hospital Clínic, Barcelona, Spain. mramos@clinic.ub.es
Robles
Angel
A
Brito-Zerón
Pilar
P
Nardi
Norma
N
Nicolás
José M
JM
Forns
Xavier
X
Plaza
Joan
J
Yagüe
Jordi
J
Sánchez-Tapias
Jose M
JM
Font
Josep
J
D002493:Central Nervous System Diseases / Q000175:diagnosis / Q000209:etiology; D003449:Cryoglobulinemia / Q000150:complications / Q000175:diagnosis* / Q000276:immunology*; D017809:Fatal Outcome; D005260:Female; D005921:Glomerulonephritis / Q000175:diagnosis / Q000209:etiology; D006470:Hemorrhage / Q000175:diagnosis / Q000209:etiology; D006801:Humans; D008171:Lung Diseases / Q000175:diagnosis / Q000209:etiology; D008297:Male; D008875:Middle Aged; D051437:Renal Insufficiency / Q000175:diagnosis / Q000209:etiology; D015996:Survival Rate; D014657:Vasculitis / Q000150:complications / Q000175:diagnosis* / Q000276:immunology*