Background and Aims

The optimal management of ANCA vasculitis patients consists of accurate identification of disease phenotypes, relapses and preventing complications such as infections and cancer. In this study, we pretend to profile the patients with ANCA vasculitis in terms of clinical features with an emphasis on kidney involvement and relapse.

Methods

Patients included in the study will be individuals ≥ 18 years or older, of both sexes, followed in an outpatient internal medicine and nephrology consultation for a period of up to ten years, with an established diagnosis of ANCA associated vasculitis. Statistical analysis was performed using SPSS software.

Results

Fifty-eight patients with ANCA-associated vasculitis were followed, fifty-four with renal involvement. The majority were male (55%), age mean of 61 years. 75.9% MPO positive. 88% had biopsy confirming vasculitis. Renal biopsies were classified into four classes: most patients (50%) had crescentic class, followed by mixed (33%), sclerotic (10.4%) and focal (6.3%). Creatinine at diagnosis was higher for the crescentic and sclerotic types. 17.2% evolved to ESRD and none was submitted to transplant. 20.7% had a relapse, with a mean time between diagnosis and relapse of 4.5 years. Females, GPA and ANCA MPO were the most affected. 5.2% of patients died. 51% of the patients had an infection in the course of the disease, being a major cause of death. 6 patients were diagnosed with cancer, mainly of the skin.

Conclusions

The prognosis of ANCA-associated vasculitis has been transformed in recent years. Once it was a set of acute and fatal conditions, but these disorders are currently considered to be chronic diseases.

Background and Aims

Cryoglobulins are antibodies that precipitate at low temperatures and dissolve after rewarming.

Cryoglobulinemia refers to the presence of circulating cryoglobulins and generally leads to a systemic inflammatory syndrome characterized by fatigue, arthralgia, purpura, ulcers, neuropathy and/or glomerulonephritis.

The disease mainly involves small to medium-sized blood vessels and causes vasculitis due to cryoglobulin-containing immune complexes.

Methods

A review of current literature on the approach to diagnosis, classification and treatment of cryoglobulinemic vasculitis.

Results

Cryoglobulinemia is classified into three types (I, II and III) on the basis of immunoglobulin composition.

Predisposing conditions include lymphoproliferative, autoimmune diseases and hepatitis C virus infection.

The diagnosis of cryoglobulinemic syndrome is predominantly based on the presence of clinical features and laboratorial demonstration of serum cryoglobulins.

The treatment strategy depends on the cause of cryoglobulinemia. For patients with chronic HCV infection, antiviral therapy is indicated. Immunosuppressive or immunomodulatory therapy, including steroids, plasmapheresis and cytotoxic agents, is reserved for organ-threatening manifestations.

Conclusions

In this review, we discuss the main clinical presentations, diagnostic approach and treatment options.