Elevations of neutrophil proteinase 3 in serum of patients with Wegener's granulomatosis and polyarteritis nodosa. 1994

T J Henshaw, and C C Malone, and J E Gabay, and R C Williams
Department of Medicine, University of Florida, Gainesville.

OBJECTIVE To determine serum levels of proteinase 3 (PR3) in normal subjects and patients with Wegener's granulomatosis (WG), polyarteritis nodosa (PAN), chronic renal failure, and systemic lupus erythematosus (SLE). METHODS Serum levels of PR3 were measured by enzyme-linked immunosorbent assay employing a monospecific rabbit polyclonal antibody against PR3. RESULTS Quantifiable levels of PR3 were detected in all serum samples examined, including those of normal subjects. Marked elevations of serum PR3, which decreased with treatment, were found in patients with active WG. Patients with PAN, SLE, and chronic renal failure also showed elevated levels of PR3. CONCLUSIONS Quantitating serum PR3 may be useful in the management of patients with WG and other connective tissue diseases.

UI MeSH Term Description Entries
D008180 Lupus Erythematosus, Systemic A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys, and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow. Libman-Sacks Disease,Lupus Erythematosus Disseminatus,Systemic Lupus Erythematosus,Disease, Libman-Sacks,Libman Sacks Disease
D010488 Polyarteritis Nodosa A form of necrotizing non-granulomatous inflammation occurring primarily in medium-sized ARTERIES, often with microaneurysms. It is characterized by muscle, joint, and abdominal pain resulting from arterial infarction and scarring in affected organs. Polyarteritis nodosa with lung involvement is called CHURG-STRAUSS SYNDROME. Essential Polyarteritis,Necrotizing Arteritis,Periarteritis Nodosa,Arteritides, Necrotizing,Arteritis, Necrotizing,Essential Polyarteritides,Necrotizing Arteritides,Polyarteritides, Essential,Polyarteritis, Essential
D012016 Reference Values The range or frequency distribution of a measurement in a population (of organisms, organs or things) that has not been selected for the presence of disease or abnormality. Normal Range,Normal Values,Reference Ranges,Normal Ranges,Normal Value,Range, Normal,Range, Reference,Ranges, Normal,Ranges, Reference,Reference Range,Reference Value,Value, Normal,Value, Reference,Values, Normal,Values, Reference
D002850 Chromatography, Gel Chromatography on non-ionic gels without regard to the mechanism of solute discrimination. Chromatography, Exclusion,Chromatography, Gel Permeation,Chromatography, Molecular Sieve,Gel Filtration,Gel Filtration Chromatography,Chromatography, Size Exclusion,Exclusion Chromatography,Gel Chromatography,Gel Permeation Chromatography,Molecular Sieve Chromatography,Chromatography, Gel Filtration,Exclusion Chromatography, Size,Filtration Chromatography, Gel,Filtration, Gel,Sieve Chromatography, Molecular,Size Exclusion Chromatography
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D001323 Autoantibodies Antibodies that react with self-antigens (AUTOANTIGENS) of the organism that produced them. Autoantibody
D012697 Serine Endopeptidases Any member of the group of ENDOPEPTIDASES containing at the active site a serine residue involved in catalysis. Serine Endopeptidase,Endopeptidase, Serine,Endopeptidases, Serine
D014890 Granulomatosis with Polyangiitis A multisystemic disease of a complex genetic background. It is characterized by inflammation of the blood vessels (VASCULITIS) leading to damage in any number of organs. The common features include granulomatous inflammation of the RESPIRATORY TRACT and KIDNEYS. Most patients have measurable autoantibodies (ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES) against MYELOBLASTIN. Granulomatosis, Wegener's,Wegener Granulomatosis,Wegener's Granulomatosis,Granulomatosis with Polyangiitides,Granulomatosis, Wegener,Polyangiitides, Granulomatosis with,Polyangiitis, Granulomatosis with,with Polyangiitides, Granulomatosis,with Polyangiitis, Granulomatosis
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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