Long-term results of treatment with direct-acting antivirals, corticosteroids, plasmapheresis, and rituximab in a patient with cryoglobulinaemic vasculitis and HCV infection: a case report and review of the literature
Nephrology, Renal Unit, General Hospital of Athens “Alexandra”, Athens, Greece
Pathology, School of Medicine, National and Kapodistrian University, Athens, Greece
Radiology, Tzaneio General Hospital of Piraeus, Greece
Gastroenterology, Tzaneio General Hospital of Piraeus, Greece
Several glomerular diseases have been associated with Hepatitis C virus (HCV) infection, including mixed cryoglobulinaemia syndrome (MCS), which is a systemic vasculitis. In its severe form, therapy should be started immediately. There are still questions concerning the optimum immunosuppressive regimen, the timing of administration of drugs, and the long-term efficacy of newer anti-viral drugs, especially in patients with renal involvement.
We present a case of a 51-year-old female patient who presented to the emergency department with weakness, leg oedema, palpable purpura in lower legs (Figure 1), and reduced urine output. At initial presentation, the patient was found to have significant pericardial effusion and reduced ejection fraction, peripheral neuropathy, pulmonary fibrosis, and ground glass opacities. A complete blood count showed mild anaemia: the haemoglobin level was 10 g/dl, with a white blood cell count of 6500/µl (with normal fractions) and a platelet count of 250,000/µl. The serum creatinine level was elevated (2.3 mg/dl). From her past medical history, she did not report any other conditions. Urine sediment was active and 24 h urine protein was 3.1 g. Immunological work-up for ANCA vasculitis or systemic lupus erythematosus (SLE) was negative, but the patient was found hypocomplementemic and hypoglobulinaemic with positive rheumatoid factor (RF). Immunofixation of serum and urine revealed monoclonic IgMk. Cryoglobulins of type II (monoclonal IgM and polyclonal IgG) were positive with high burden (cryocrit 25%). Further evaluation revealed that the patient was HCV-RNA positive, genotype 4, with high viral load (4.98 × 106 IU/ml). Liver function tests were normal, and liver stiffness was compatible with METAVIR score F3.
The patient underwent renal biopsy, and the results showed glomerulonephritis with membranoproliferative pattern (Figure 2). Endocapillary hyaline thrombi were depicted with hyper-microscopic features suggestive of cryoglobulins. Immunofluorescence showed positivity for IgG/IgM/C3/C1g and both light chains k and l (Figure 3). The work up for non-Hodgkin lymphoma or lymphoproliferative disease was negative.
The clinical manifestations suggested a severe...
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