title: ANCA-positive vasculitis as a secondary autoimmune disease after autologous stem cell transplantation for systemic sclerosis: a case report.
abstract: Autologous stem cell transplantation (SCT) is increasingly used to treat autoimmune diseases (AD), in particular systemic sclerosis (SSc). Secondary autoimmune diseases are a known complication after autologous stem cell transplantations for any cause. A 43-year-old man had received an autologous stem cell transplantation for an aggressive diffuse cutaneous SSc. After mobilisation with cyclophosphamide and Granulocyte-Colony-Stimulating Factor stem cells were CD34-selected. The patient received a conditioning regimen with cyclophosphamide and Antithymocyte globulin. He had an excellent response with the modified Rodnan Skin Score decreasing from 34 to 3. One year and 4 months after SCT mild erythrocyturia without acanthocytes and proteinuria were seen for the first time on routine urinalysis. During the following year erythrocyturia increased to 131 erythrocytes /μl and protein excretion to 628 mg/g creatinine. At that time, acanthocytes of 25% finally could be detected. Due to the clearly nephritic constellation in urinalysis a renal biopsy was performed, which revealed mild global and focal-segmental sclerosing and focal-segmental proliferative glomerulonephritis without any signs of a IgA-nephropathy. The result was compatible with a renal manifestation of a small-vessel vasculitis. During the following laboratory workup ANCA of a perinuclear pattern with specificity for myeloperoxidase in high titers could be detected. Therefore the diagnosis of a p-ANCA-positive glomerulonephritis was established. As treatment, the patient received Rituximab, which turned out to be effective. We provide the first report of a patient who developed a p-ANCA-associated vasculitis after autologous stem cell transplantation for an autoimmune disease, namely systemic sclerosis.
authors.affiliation: Department of Internal Medicine 2, University Hospital, Wuerzburg, Germany. schmalzing_m@ukw.de.
authors.forename: M
authors.initials: M
authors.lastname: Schmalzing
authors.forename: K
authors.initials: K
authors.lastname: Amann
authors.forename: H-P
authors.initials: HP
authors.lastname: Tony
mesh_terms: D000328:Adult; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000150:complications / Q000473:pathology*; D005921:Glomerulonephritis / Q000150:complications / Q000473:pathology*; D006801:Humans; D008297:Male; D045743:Scleroderma, Diffuse / Q000150:complications / Q000628:therapy*; D033581:Stem Cell Transplantation; D014182:Transplantation, Autologous