Biomaterial Database

Vasculitis in primary vasculitides, granulomatoses, and connective tissue diseases. 1983

R T McCluskey, and R Fienberg

Problems with the classification and diagnosis of vasculitides are discussed. The evidence related to the pathogenetic importance of immune complexes in vasculitis, both in experimental models and human disease, is reviewed. The finding, by immunofluorescence, of immunoglobulins and complement components in vessel walls has provided indirect evidence of a role of immune complexes in certain forms of human vasculitis. However, specific antigens have been demonstrated in very few instances, notably in some patients with hepatitis B infection. In most forms of human vasculitis there is no information about causative factors. The widely held belief that therapeutic drugs cause an appreciable proportion of cases of human vasculitis appears unfounded.

UI	MeSH Term	Description	Entries
D007105	Immune Complex Diseases	Group of diseases mediated by the deposition of large soluble complexes of antigen and antibody with resultant damage to tissue. Besides SERUM SICKNESS and the ARTHUS REACTION, evidence supports a pathogenic role for immune complexes in many other IMMUNE SYSTEM DISEASES including GLOMERULONEPHRITIS, systemic lupus erythematosus (LUPUS ERYTHEMATOSUS, SYSTEMIC) and POLYARTERITIS NODOSA.	Hypersensitivity, Type III,Type III Hypersensitivity,Disease, Immune Complex,Diseases, Immune Complex,Hypersensitivities, Type III,Immune Complex Disease,Type III Hypersensitivities
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
D011695	IgA Vasculitis	A systemic non-thrombocytopenic purpura caused by HYPERSENSITIVITY VASCULITIS and deposition of IGA-containing IMMUNE COMPLEXES within the blood vessels throughout the body, including those in the kidney (KIDNEY GLOMERULUS). Clinical symptoms include URTICARIA; ERYTHEMA; ARTHRITIS; GASTROINTESTINAL HEMORRHAGE; and renal involvement. Most cases are seen in children after acute upper respiratory infections.	Allergic Purpura,Anaphylactoid Purpura,Henoch Purpura,Henoch-Schoenlein Purpura,Purpura Hemorrhagica,Purpura, Nonthrombocytopenic,Purpura, Schoenlein-Henoch,Rheumatoid Purpura,Schoenlein-Henoch Purpura,Vasculitis, Hemorrhagic,Henoch Schonlein Purpura,Henoch-Schonlein Purpura,Purpura, Nonthrombopenic,Purpura, Schonlein-Henoch,Hemorrhagic Vasculitis,Hemorrhagica, Purpura,Henoch Schoenlein Purpura,Henoch Schonlein Purpuras,Henoch-Schonlein Purpuras,Nonthrombocytopenic Purpura,Nonthrombopenic Purpura,Nonthrombopenic Purpuras,Purpura, Allergic,Purpura, Anaphylactoid,Purpura, Henoch,Purpura, Henoch Schonlein,Purpura, Henoch-Schoenlein,Purpura, Henoch-Schonlein,Purpura, Rheumatoid,Purpura, Schoenlein Henoch,Purpura, Schonlein Henoch,Purpuras, Henoch Schonlein,Purpuras, Henoch-Schonlein,Purpuras, Nonthrombopenic,Purpuras, Schonlein-Henoch,Schoenlein Henoch Purpura,Schonlein Purpura, Henoch,Schonlein Purpuras, Henoch,Schonlein-Henoch Purpura,Schonlein-Henoch Purpuras,Vasculitis, IgA
D003095	Collagen Diseases	Historically, a heterogeneous group of acute and chronic diseases, including rheumatoid arthritis, systemic lupus erythematosus, progressive systemic sclerosis, dermatomyositis, etc. This classification was based on the notion that "collagen" was equivalent to "connective tissue", but with the present recognition of the different types of collagen and the aggregates derived from them as distinct entities, the term "collagen diseases" now pertains exclusively to those inherited conditions in which the primary defect is at the gene level and affects collagen biosynthesis, post-translational modification, or extracellular processing directly. (From Cecil Textbook of Medicine, 19th ed, p1494)	Collagen Disease,Disease, Collagen,Diseases, Collagen
D003165	Complement System Proteins	Serum glycoproteins participating in the host defense mechanism of COMPLEMENT ACTIVATION that creates the COMPLEMENT MEMBRANE ATTACK COMPLEX. Included are glycoproteins in the various pathways of complement activation (CLASSICAL COMPLEMENT PATHWAY; ALTERNATIVE COMPLEMENT PATHWAY; and LECTIN COMPLEMENT PATHWAY).	Complement Proteins,Complement,Complement Protein,Hemolytic Complement,Complement, Hemolytic,Protein, Complement,Proteins, Complement,Proteins, Complement System
D003449	Cryoglobulinemia	A condition characterized by the presence of abnormal quantities of CRYOGLOBULINS in the blood. Upon cold exposure, these abnormal proteins precipitate into the microvasculature leading to restricted blood flow in the exposed areas.	Cryoglobulinemias
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D001172	Arthritis, Rheumatoid	A chronic systemic disease, primarily of the joints, marked by inflammatory changes in the synovial membranes and articular structures, widespread fibrinoid degeneration of the collagen fibers in mesenchymal tissues, and by atrophy and rarefaction of bony structures. Etiology is unknown, but autoimmune mechanisms have been implicated.	Rheumatoid Arthritis
D013577	Syndrome	A characteristic symptom complex.	Symptom Cluster,Cluster, Symptom,Clusters, Symptom,Symptom Clusters,Syndromes