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Proteinase 3-antineutrophil cytoplasmic antibody-(PR3-ANCA) positive necrotizing glomerulonephritis after restarting sulphasalazine treatment. 2009

N Miura, and R Aoyama, and W Kitagawa, and H Yamada, and K Nishikawa, and H Imai
Division of Nephrology and Rheumatology, Department of Internal Medicine, Aichi Medical University School of Medicine, Aichi, Japan.

A 59-year-old woman with ulcerative colitis developed red eyes, pleural effusion, eosinophilia and urinary abnormalities after restarting of sulphasalazine treatment. Light microscopy of a kidney biopsy revealed segmental necrotizing glomerulonephritis without deposition of immunoglobulin or complement. Proteinase 3-antineutrophil cytoplasmic antibody (PR3-ANCA) titer was elevated at 183 ELISA units (EU) in sera (normal range less than 10 EU), myeloperoxidase-ANCA was negative. PR3-ANCA titer was 250 and 1,070 EU in pleural effusions on right and left side, respectively. Although cessation of sulphasalazine treatment resulted in improvements in fever, red eyes, chest pain, titer of C-reactive protein and volume of the pleural effusions, we initiated steroid therapy, because PR3-ANCA titer rose to 320 EU, eosinophil count increased to 1,100 cells/microl, and the pleural effusion remained. One month after steroid therapy, the pleural effusion disappeared, and PR3-ANCA titer normalized 3 months later. This case suggests that sulphasalazine can induce PR3-ANCA-positive necrotizing glomerulonephritis.

UI MeSH Term Description Entries
D007673 Kidney Cortex Necrosis Death of cells in the KIDNEY CORTEX, a common final result of various renal injuries including HYPOXIA; ISCHEMIA; and drug toxicity. Renal Cortical Necrosis,Glomerular Necrosis,Renal Tubule Necrosis,Necrosis, Glomerular,Necrosis, Kidney Cortex,Necrosis, Renal Cortical,Necrosis, Renal Tubule
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D003093 Colitis, Ulcerative Inflammation of the COLON that is predominantly confined to the MUCOSA. Its major symptoms include DIARRHEA, rectal BLEEDING, the passage of MUCUS, and ABDOMINAL PAIN. Colitis Gravis,Idiopathic Proctocolitis,Inflammatory Bowel Disease, Ulcerative Colitis Type,Ulcerative Colitis
D005260 Female Females
D005765 Gastrointestinal Agents Drugs used for their effects on the gastrointestinal system, as to control gastric acidity, regulate gastrointestinal motility and water flow, and improve digestion. Digestants,Gastric Agents,Gastric Drugs,Gastrointestinal Drugs,Agents, Gastric,Agents, Gastrointestinal,Drugs, Gastric,Drugs, Gastrointestinal
D005923 Glomerulosclerosis, Focal Segmental A clinicopathological syndrome or diagnostic term for a type of glomerular injury that has multiple causes, primary or secondary. Clinical features include PROTEINURIA, reduced GLOMERULAR FILTRATION RATE, and EDEMA. Kidney biopsy initially indicates focal segmental glomerular consolidation (hyalinosis) or scarring which can progress to globally sclerotic glomeruli leading to eventual KIDNEY FAILURE. Glomerulonephritis, Focal Sclerosing,Hyalinosis, Segmental Glomerular,Focal Segmental Glomerulosclerosis,Glomerulosclerosis, Focal,Hyalinosis, Segmental,Segmental Glomerular Hyalinosis,Focal Glomerulosclerosis,Focal Sclerosing Glomerulonephritides,Focal Sclerosing Glomerulonephritis,Glomerular Hyalinosis, Segmental,Glomerulonephritides, Focal Sclerosing,Sclerosing Glomerulonephritides, Focal,Sclerosing Glomerulonephritis, Focal,Segmental Glomerulosclerosis, Focal,Segmental Hyalinosis
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D012460 Sulfasalazine A drug that is used in the management of inflammatory bowel diseases. Its activity is generally considered to lie in its metabolic breakdown product, 5-aminosalicylic acid (see MESALAMINE) released in the colon. (From Martindale, The Extra Pharmacopoeia, 30th ed, p907) Salicylazosulfapyridine,Asulfidine,Azulfadine,Azulfidine,Azulfidine EN,Colo-Pleon,Pleon,Pyralin EN,Salazopyrin,Salazosulfapyridine,Sulfasalazin medac,Sulfasalazin-Heyl,Sulfasalazine FNA,Sulphasalazine,Ucine,Ulcol,ratio-Sulfasalazine,Colo Pleon,Sulfasalazin Heyl,ratio Sulfasalazine
D053146 Myeloblastin A polymorphonuclear leukocyte-derived serine protease that degrades proteins such as ELASTIN; FIBRONECTIN; LAMININ; VITRONECTIN; and COLLAGEN. It is named for its ability to control myeloid cell growth and differentiation. Neutrophil Proteinase 3,Wegener Autoantigen,Azurophil Granule Protein 7,C-ANCA Antigen,Leukocyte Proteinase 3,Myeloblastin, Human,N-Proteinase 4,Neutrophil Proteinase 3, Human,Neutrophil Proteinase 4,PRTN3 Gene Protein,Proteinase 3,Antigen, C-ANCA,Autoantigen, Wegener,C ANCA Antigen,Human Myeloblastin,N Proteinase 4,Protein, PRTN3 Gene,Proteinase 3, Leukocyte,Proteinase 3, Neutrophil,Proteinase 4, Neutrophil
D019268 Antibodies, Antineutrophil Cytoplasmic Autoantibodies directed against cytoplasmic constituents of POLYMORPHONUCLEAR LEUKOCYTES and/or MONOCYTES. They are used as specific markers for GRANULOMATOSIS WITH POLYANGIITIS and other diseases, though their pathophysiological role is not clear. ANCA are routinely detected by indirect immunofluorescence with three different patterns: c-ANCA (cytoplasmic), p-ANCA (perinuclear), and atypical ANCA. ANCA,Anti-Neutrophil Cytoplasmic Autoantibodies,Anti-Neutrophil Cytoplasmic Autoantibody,Antineutrophil Cytoplasmic Antibodies,Neutrophil Cytoplasmic Autoantibodies,Neutrophil Cytoplasmic Autoantibody,c-ANCA,p-ANCA,Anti-Neutrophil Cytoplasmic Antibodies,Anti-Neutrophil Cytoplasmic Antibody,Antineutrophil Cytoplasmic Antibody,Anti Neutrophil Cytoplasmic Antibodies,Anti Neutrophil Cytoplasmic Antibody,Anti Neutrophil Cytoplasmic Autoantibodies,Anti Neutrophil Cytoplasmic Autoantibody,Antibody, Anti-Neutrophil Cytoplasmic,Antibody, Antineutrophil Cytoplasmic,Autoantibody, Anti-Neutrophil Cytoplasmic,Autoantibody, Neutrophil Cytoplasmic,Cytoplasmic Antibody, Anti-Neutrophil,Cytoplasmic Antibody, Antineutrophil,Cytoplasmic Autoantibody, Anti-Neutrophil,Cytoplasmic Autoantibody, Neutrophil,c ANCA,p ANCA

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