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AND THE ANSWER IS . . .
Discussion
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AND THE ANSWER IS . . .
1-b; 2-e; 3-d; 4-c; 5-a Discussion
1. Serum antibodies against neutrophil cytoplasm components with a diffuse immunofluorescent staining pattern on ethanol-fixed smears of neutrophils (c-ANCA) are typically found in Wegener's Granulomatosis, particularly those cases with active extra-respiratory as well as respiratory involvement (1). The c-ANCA are thought to be directed predominantly against Proteinase 3, a tryptic type of serine proteinase. However, the p-ANCA, not the c-ANCA, pattern is sometimes seen in Wegener's. Also, the c-ANCA is occasionally seen in some types of primary vasculitis (2).
2. Antibodies against a E2 sub-unit of the pyruvate dehydrogenase complex are responsible for anti-mitochondrial antibody binding, found most commonly in IBC (3). Levels of these antibodies are increased in only a small minority of autoimmune hepatitis and not in biliary cirrhosis secondary to extra hepatic mechanical obstruction.
3. Rheumatoid factor levels, the immunologic test most commonly identified with rheumatoid arthritis (AR) are present in about 70% of RA cases but are also present in a wide variety of other disorders (4). The anti-CCP antibodies are present in about 50-60% of RA, but are relatively specific for RA(>90%) (5).
4. Auto-immune hepatitis, Type 1 is characterized by anti-smooth muscle antibodies (>90), anti-nuclear antibodies and sometimes lupus-like extra hepatic manifestations (joints, serositis, rash) (6). The anti-smooth muscle antibody is present in only a small minority of chronic viral hepatitis cases.
5. The CREST (calinosis, Raynaud's, esophageal dysmobility, sclerodactyly, telangiectasia) limited presentation of scleroderma is characterized by the presence of anti-kinetochore antibody which gives an anti-centromere antibody staining pattern in any dividing target cells in the substrate of an anti-nuclear antibody test (7). Such antibodies are found much less frequently in the systemic sclerosis presentation of scleroderma, characterized by more extensive skin involvement, pulmonary and sometimes cardiac and/or renal involvement.
References
1. Arthritis Rheum. 2003;48:2299-309
2. Am J Clin Pathol 1999 ;111:363-9
3. Immunol Rev 2000 ;174:226-37
4. Scand J Rheumatol Suppl 1988;75:300-8
5. Rheumatology (Oxford). 2003 ;42:677-80.
6. Eur J Intern Med 2002 ;13:293-303
7. Arthritis Res Ther. 2003;5:80-93
