ABSTRACT
OBJECTIVES: We aimed to elucidate the frequency and associations of MPO-ANCA positivity in patients without ANCA-associated vasculitis (AAV), in a large urban, multi-ethnic teaching hospital. METHODS: Retrospective review of 200 patients identified as MPO-ANCA positive over a five-year period at Royal Free Hospital, London, UK. RESULTS: The incidence of anti-MPO positivity in patients without AAV was 39.5%. Gastrointestinal tract disorders, infections and other connective tissue disorders made up the majority of diagnoses, and there was a higher incidence of other concomitant autoantibodies compared to the group with known AAV. Renal disease was common in non-vasculitic patients with anti-MPO antibody positivity (occurring in 48%), the majority of whom went on to renal biopsy to exclude vasculitic involvement. CONCLUSIONS: The high incidence of MPO-ANCA positivity in patients with non-vasculitic conditions highlights the need for careful clinical correlation and confirmatory tissue diagnosis.
Subject(s)
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis , Antibodies, Antineutrophil Cytoplasmic/analysis , Peroxidase , Adult , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/blood , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/diagnosis , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/immunology , Female , Humans , London , Male , Peroxidase/blood , Peroxidase/immunology , Retrospective StudiesABSTRACT
BACKGROUND: Morbidity associated with monoclonal gammopathy of renal significance is high due to the severe renal lesions and the associated systemic alterations. Accordingly, early diagnosis is fundamental, as is stopping the clonal production of immunoglobulins using specific chemotherapy. CASE PRESENTATION: A 75-year-old man with chronic renal disease of unknown origin since 2010 experienced rapid worsening of renal function over a period of 6 mos. Bone marrow biopsy showed monoclonal gammopathy of undetermined significance. Kidney biopsy showed the presence of C3 glomerulonephritis, with exclusive deposits of C3 visible on immunofluorescence and a membranoproliferative pattern on light microscopy. Skin biopsy showed endothelial deposition of complement. Given both the renal and cutaneous involvement the patient was considered to have monoclonal gammopathy of renal significance. We considered an underlying pathogenic mechanism for the renal alteration secondary to activation of the alternative complement pathway by the anomalous immunoglobulin. Despite treatment with plasmapheresis, bortezomib and steroids, advanced chronic kidney disease developed. CONCLUSIONS: The possible underlying cause of the monoclonal gammopathy of renal significance suggests that monoclonal gammopathy should be considered in adult patients with membranoproliferative glomerulonephritis.
