Treatment of idiopathic mixed cryoglobulinemia with alpha interferon

doi:10.1016/0002-9343(87)90904-1

The American Journal of Medicine

Volume 83, Issue 4, October 1987, Pages 726-730

https://doi.org/10.1016/0002-9343(87)90904-1 Get rights and content

Abstract

Seven patients with type II idiopathic mixed cryoglobulinemia were treated with recombinant human leukocyte interferon (alpha interferon). In all of them, a conspicuous reduction of circulating cryoglobulins was noted, together with a definite, remarkable improvement of the clinical pattern. The immunologic parameters (natural killer cell activity and phagocytosis, among others) improved as well; side effects were usually mild and transient. Increases in the cryoglobulin level occurred in a few cases, but they were at least partly sensitive to readministration of alpha interferon treatment. The favorable results obtained in these cases of idiopathic cryoglobulinemia seem to be consistent and prolonged.

References (11)

M Meltzer et al.
Cryoglobulinemia
JC Brouet et al.
Biological and clinical significance of cryoglobulins
Am J Med
(1974)
F Dammacco et al.
Cryoglobulinemia in four sisters
Acta Haematol (Basel)
(1978)
L Bonomo et al.
IgG-IgM complexes in cryoglobulinemia with monoclonal or polyclonal antigammaglobulin IgM component
M Casato et al.
Interferon treatment in idiopathic mixed cryoglobulinemia

There are more references available in the full text version of this article.

Cited by (163)

Vasculitides and Hepatitis C Infection
2015, Infection and Autoimmunity
Chronic infection due to the hepatitis C virus (HCV) is among the most common etiologic factors of vasculitis in humans. In less than 5% of cases, long-term infection is associated with small-vessel vasculitis (HCV-associated cryoglobulinemic vasculitis (CV)) or more rarely with medium-size vasculitis (polyarteritis nodosa-like). In recent years, significant advances have been made in our understanding of the pathogenesis of HCV-associated CV, as well as its long-term prognosis. Furthermore, new immunosuppressive agents such as rituximab (a B-cell-depleting agent) have been shown to be efficacious with limited toxicity, and these agents appear to provide a promising alternative to currently available immunosuppressives for the treatment of HCV-associated CV. In this chapter, the pathogenesis, clinical features and recent therapeutic options for HCV-associated CV and medium-size vasculitis are presented.
Hepatitis C treatment in special patient groups
2014, Gastroenterologia y Hepatologia
El plan de tratamiento de la hepatitis crónica C en las poblaciones especiales varía en función de la comorbilidad y las evidencias de tratamiento existentes. En los pacientes con coinfección por virus de la hepatitis C y virus de la inmunodeficiencia humana, los resultados del tratamiento con biterapia (interferón pegilado más ribavirina) son pobres. En los pacientes infectados por virus de genotipo 1, la terapia triple (biterapia más boceprevir o telaprevir) ha duplicado la tasa de respuesta, pero los inhibidores de la proteasa pueden interactuar con algunos fármacos antirretrovirales y provocan más efectos adversos. Estos inconvenientes no los presentan los nuevos antivirales directos de segunda generación. En los pacientes candidatos a trasplante hepático o portadores ya de un trasplante hepático, la mejor opción terapéutica actual es combinar los nuevos antivirales, con o sin ribavirina y sin interferón. El tratamiento de los enfermos en hemodiálisis por enfermedad renal crónica continúa siendo la biterapia (en muchos casos, con dosis reducidas de interferón pegilado y ribavirina), pues no se dispone todavía de suficiente información sobre la triple terapia ni los nuevos antivirales. En la crioglobulinemia mixta, aunque existe poca experiencia, la triple terapia parece ser superior a la biterapia y puede rescatar pacientes que no responden a la biterapia; pero siempre debe decidirse si el tratamiento antiviral debe asociarse o posponerse al tratamiento inmunosupresor.
The treatment plan for chronic hepatitis C in special populations varies according to comorbidity and the current evidence on treatment. In patients with hepatitis C virus and HIV coinfection, the results of dual therapy (pegylated interferon plus ribavirin) are poor. In patients with genotype 1 infection, triple therapy (dual therapy plus boceprevir or telaprevir) has doubled the response rate, but protease inhibitors can interact with some antiretroviral drugs and provoke more adverse effects.
These disadvantages are avoided by the new, second-generation, direct-acting antiviral agents. In patients who are candidates for liver transplantation or are already liver transplant recipients, the optimal therapeutic option at present is to combine the new antiviral agents, with or without ribavirin and without interferon. The treatment of patients under hemodialysis due to chronic renal disease continues to be dual therapy (often with reduced doses of pegylated interferon and ribavirin), since there is still insufficient information on triple therapy and the new antiviral agents. In mixed cryoglobulinemia, despite the scarcity of experience, triple therapy seems to be superior to dual therapy and may be used as rescue therapy in non-responders to dual therapy. However, a decision must always be made on whether antiviral treatment should be used concomitantly or after immunosuppressive therapy.
Renal involvement in HCV-related vasculitis
2013, Clinics and Research in Hepatology and Gastroenterology
Citation Excerpt :
In severe systemic disease, patients were treated aggressively with high-dose corticosteroids, immunsuppressants (mainly cyclophosphamide) and/or plasmapheresis. Interestingly and in the absence of evidence of a viral etiology, the empiric use of IFN-α, as an antiproliferative agent, was thought to be an effective treatment for CryoVas [44]. With the discovery of HCV as the etiologic agent for most cases of mixed CryoVas, new opportunities and problems for crafting therapy have emerged, according to the HCV status.
Renal involvement has been frequently reported in the setting of hepatitis C virus (HCV) infection. The most common renal pathology associated with chronic HCV infection is type I membranoproliferative glomerulonephritis associated with type II mixed cryoglobulinemia, while membranoproliferative glomerulonephritis without cryoglobulinemia and membranous nephropathy were less frequently reported. Rarely, focal segmental glomerulosclerosis, fibrillary and immunotactoid glomerulopathies, and thrombotic microangiopathies were described during the course of HCV infection. In the present review, we have focused on renal involvement in HCV-related vasculitis.
Recommendations for the management of mixed cryoglobulinemia syndrome in hepatitis C virus-infected patients
2011, Autoimmunity Reviews
Citation Excerpt :
Since the discovery of the association between HCV and MCS, many studies have assessed the efficacy of antiviral therapy [13]. Interestingly, because of its antiproliferative and immunomodulatory effects, interferon-α (IFN) was successfully used to treat MC even before the identification of HCV [14,15]. The antiviral treatment of MCS essentially followed the evolution of chronic hepatitis C treatment, but the studies are difficult to compare because of the heterogeneity of treatment regimens, patient selection, response evaluations, and follow-up [16].
The objective of this review was to define a core set of recommendations for the treatment of HCV-associated mixed cryoglobulinemia syndrome (MCS) by combining current evidence from clinical trials and expert opinion.
Expert physicians involved in studying and treating patients with MCS formulated statements after discussing the published data. Their attitudes to treatment approaches (particularly those insufficiently supported by published data) were collected before the consensus conference by means of a questionnaire, and were considered when formulating the statements.
An attempt at viral eradication using pegylated interferon plus ribavirin should be considered the first-line therapeutic option in patients with mild–moderate HCV-related MCS. Prolonged treatment (up to 72 weeks) may be considered in the case of virological non-responders showing clinical and laboratory improvements. Rituximab (RTX) should be considered in patients with severe vasculitis and/or skin ulcers, peripheral neuropathy or glomerulonephritis. High-dose pulsed glucocorticoid (GC) therapy is useful in severe conditions and, when necessary, can be considered in combination with RTX; on the contrary, the majority of conference participants discouraged the chronic use of low–medium GC doses. Apheresis remains the elective treatment for severe, life-threatening hyper-viscosity syndrome; its use should be limited to patients who do not respond to (or who are ineligible for) other treatments, and emergency situations. Cyclophosphamide can be considered in combination with apheresis, but the data supporting its use are scarce. Despite the limited available data, colchicine is used by many of the conference participants, particularly in patients with mild–moderate MCS refractory to other therapies. Careful monitoring of the side effects of each drug, and its effects on HCV replication and liver function tests is essential. A low-antigen-content diet can be considered as supportive treatment in all symptomatic MCS patients. Although there are no data from controlled trials, controlling pain should always be attempted by tailoring the treatment to individual patients on the basis of the guidelines used in other vasculitides.
Although there are few controlled randomised trials of MCS treatment, increasing knowledge of its pathogenesis is opening up new frontiers. The recommendations provided may be useful as provisional guidelines for the management of MCS.
Therapy for hepatitis C virus-related cryoglobulinemic vasculitis
2013, New England Journal of Medicine
Citation Excerpt :
The effectiveness of interferon alfa in the treatment of patients with mixed cryoglobulinemia was observed empirically in 198735; these observations were confirmed and extended in 1991.36
Cryoglobulins are present in about a quarter of patients with hepatitis C; in some, cryoglobulinemia can become symptomatic or even life-threatening. This review summarizes evolving therapy for the disorder, including new therapeutic options that are becoming available.
Cryoglobulins are immunoglobulins characterized by their insolubility at low temperatures and their dissolution after rewarming.^1,² On the basis of their immunochemical composition, cryoglobulins are classified as either single (type I), consisting of a monoclonal immunoglobulin, or mixed, comprising two or more immunoglobulin isotypes, with (type II) or without (type III) a monoclonal component.³ For many years, mixed cryoglobulinemia was referred to as “essential” because of its undefined cause. In the early 1990s, it became evident that more than 90% of patients with mixed cryoglobulinemia were infected with hepatitis C virus (HCV).4, 5, 6, 7 The pathophysiology of mixed cryoglobulinemia, as it . . .
Hepatitis C virus-related cryoglobulinemic vasculitis
2021, Minerva Medica

View all citing articles on Scopus

^☆: This work was supported by contributions from Consiglio Nazionale delle Ricerche, n°86.00565.44.

View full text

Treatment of idiopathic mixed cryoglobulinemia with alpha interferon☆

Abstract

Am J Med

Cryoglobulinemia in four sisters

Acta Haematol (Basel)

IgG-IgM complexes in cryoglobulinemia with monoclonal or polyclonal antigammaglobulin IgM component

Interferon treatment in idiopathic mixed cryoglobulinemia