Non HCV-related infectious cryoglobulinemia vasculitis: Results from the French nationwide CryoVas survey and systematic review of the literature.

In patients with infectious cryoglobulinemia vasculitis (CryoVas) in the absence of hepatitis C virus infection, data on presentation, therapeutic management and outcome are lacking. We conducted a nationwide survey that included patients with HCV-negative CryoVas. We describe here the presentation, therapeutic management and outcome of 18 patients with non-HCV infectious CryoVas and 27 additional patients identified form a systematic review of the literature. We included 18 patients, mean age 57.9±13.5 years. Infectious causes were viral infections in 8 patients [hepatitis B virus (HBV) in 4, and cytomegalovirus, Epstein Barr virus, parvovirus B19 and human immunodeficiency virus in one case each], pyogenic bacterial infection in 6 patients, parasitic infection in 2 patients, and leprosy and candidiasis in one case each. Baseline manifestations were purpura (78%), glomerulonephritis (28%), arthralgia (28%), peripheral neuropathy (22%), skin necrosis (22%), cutaneous ulcers (17%), and myalgia (11%). Cryoglobulinemia was type II in 2/3 of cases. Most cases received specific anti-infectious therapy as first-line therapy, sometimes associated with corticosteroids, achieving sustained remission in the majority of cases. Refractory or relapsing patients, frequently related to HBV infection, showed a complete remission after rituximab in addition to antiviral therapy. In contrast, corticosteroids and/or immunosuppressive agents used in the absence of anti-infectious agents were frequently associated with refractory CryoVas. Viral and pyogenic bacterial infections represent the main causes of non-HCV infectious CryoVas. Antimicrobial therapy is commonly associated with sustained remission. Immunosuppressive agents should be considered only as a second-line option in patients with refractory vasculitis.

Infectious neuropathies.

Peripheral neuropathies can result from several infective agents, ranging from viruses, especially retroviruses, to parasites and bacilli. Leprosy, which often is considered a disorder of the past, still is common in dome geographic areas, especially in Africa, South America, and Asia. An increasing number of cases of neuropathies occurs in patients who have HIV or Lyme disease. The important point is that all these neuropathies are treatable and often preventable.

An uncommon presentation of an uncommon disease: leprosy in a heart transplant recipient.

The effect of solid-organ transplantation on the acquisition, presentation and course of leprosy is unknown. We present a case of leprosy in a heart transplant recipient with multiple unique features possibly attributed to altered immune function.

The pathology of HIV infection.

The acquired immunodeficiency syndrome (AIDS) is the result of a human immunodeficiency virus (HIV) infection damaging the cell-mediated immune system. A wide range of opportunistic infections (OI) and tumours develop; additionally, HIV directly damages some organs. The patterns of opportunistic diseases (OD) are different in different parts of the world, depending on the local prevalence of latent and acquired infections and on the survival of HIV-infected patients. OD patterns change as people migrate. Recently introduced highly active anti-retroviral chemotherapy prevents many of the common OIs, but also introduces a new range of toxic pathological damage. Longer survival permits development of new HIV-related diseases. The pathology of HIV/AIDS is not static but changing.

Infective neuropathies.

Infective neuropathies encompass neuropathies that are among the most common in the world. Retroviral infection, which includes infection with the human immunodeficiency virus, has now spread worldwide. This virus is responsible for a number of disabling peripheral neuropathies, either from the immune reaction that follows penetration of the virus into nervous system of the host, or by opportunistic infection secondary to the major cellular immunodeficit induced by gradual destruction of lymphocytes bearing the CD4 antigen on their surface. In the other class of retroviruses, human T lymphotrophic viruses (HTLV), which are responsible for the HTLV-I-associated myelopathy or tropical spastic paraparesis, peripheral nerve involvement and inflammatory myopathy are less common and milder than in HIV infection. Leprosy continues to pose problems concerning the understanding of the immune mechanisms that lead to the various patterns of nerve lesions encountered in this condition. Chagas' disease, which is due to infection with Trypanosoma cruzi, affects more than 15 million people in Latin America. It is accompanied by mostly subclinical peripheral nerve involvement and by cardiac manifestations from lesions of the autonomic nervous system and cardiac muscle.