Primary systemic vasculitis

Lane, S.E. et al., Are environmental factors important in primary systemic vasculitis A case-control study, Arthritis Rheum., 48, 814, 2003. 

Anti-MPO antibodies have been studied in subjects with propylthiouracil-induced vasculitis or primary systemic vasculitis there was a potential difference in epitope recognition (52). 

Savage COS, Harper L, Adu D (1997). Primary systemic vasculitis. Lancet 349 553-558... 

Jayne D. How to induce remission in primary systemic vasculitis. Best Pract Res Clin Rheumatol 2005 19(2) 293-305. 

Zhang L, Ja3Tie DR, Zhao MH, et al. Distribution of MHC class II alleles in primary systemic vasculitis. Kidney Int 1995 47 294-298. 

Salvarani C, Brown RDJ, Calamia KT, Christianson TJH, Weigand SD, MiUer DV, Giannmi C, Meschia JF, Huston J3, Hunder GG Primary central nervous system vasculitis analysis of 101 patients. Aim Neurol 2007 62 pp. 442-451. 

Primary angiitis of the CNS causes inflammation particularly in small leptomeningeal vessels, whereas larger vessels can also be affected. Systemic variants of vasculitis and secondary arteritis of the CNS affect mostly small or medium-sized arteries to different degrees, and occasionally the venous system is also afflicted as in Behcet disease. Segmental stenoses are frequently found, often not including bifurcations and alternating with arterial dilatations. This pattern is not definitively specific and can also be seen in atherosclerosis. Even with an optimal MRA technique, DSA still remains necessary for the depiction of tiny vessel lesions (Fig. 5.26). 

Primary vasculitis of the central nervous system Takayasu s disease Buerger s disease Malignant atrophic papulosis... 

Almost all autoimmune diseases that occur in adults disproportionately affect women. However, there is considerable variability in the extent of female predominance and no clear relation between degree of female predominance and type of disease or age at onset (Table 7). More than 85% of patients with Sjogren syndrome, systemic lupus erythematosus, systemic sclerosis, the autoimmune thyroid diseases, and primary biliary cirrhosis are female, compared with 65-75% of patients with rheumatoid arthritis and multiple sclerosis. Some diseases (e.g. diabetes mellitus type 1, ANCA-associated vasculitis, primary sclerosing cholangitis) occur more often in males than in females. 

D-penicillamine is so named because it was first isolated as an amine, from the degradation products of penicillin by Abraham et al [87]. Later studies showed the characteristic chemical behavior of D-penicillamine which involve three types of reactions, formation of disulphide links, formation of thiazolidine rings, and formation of metal complexes and chelates [67]. It was first used in 1956 in the treatment of Wilson s disease [88]. D-penicillamine has since been used in the treatment of many diseases, such as cystinuria [89], rheumatoid arthritis [90-92], systemic sclerosis [93], primary bdiary cirrhosis [94], heavy metal poisoning due to lead [95], cadmium [%], and mercury [97], and hyperviscosity syndrome [99]. In rheumatoid arthritis, D-peni-cdlamine has been widely accepted as an effective second line treatment. Despite of its effectiveness, it causes many adverse effects, such as skin rashes [99,100], taste abnormalities [100,101], hepatic dysfunction [102-104], gastrointestinal toxiciiy [99,105], proteinuria [100,106], hematuria [107, 108], thrombocytopenia [92, 109], aplastic anemia [110], lupus-like syndrome [111, 112], Goodpasture s-tike pulmonary renal syndrome [113-115], vasculitis [116,117], myasthenia gravis [118-122], polymyositis [123, 124], and dermatomyositis [125]. 

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