Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0042384 (vasculitis)
 20,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

HLA B51 specificity is strongly associated with Behcet's disease (BD) (for references see Baricordi et al., 1986), a multisystem vasculitis of unknown aetiology, for which an immunological pathogenesis has been proposed (O'Duffy et al., 1983; O'Duffy et al., 1990). Neutrophil abnormalities observed in BD patients even during clinical remission suggest prominent involvement of these phagocytic cells in the pathogenesis of the disease (Niwa et al., 1982). In order to clarify how HLA B51 antigen might confer susceptibility to BD, we have investigated neutrophil function in 13 B51-positive and 13 B51-negative healthy subjects. Lymphocyte spontaneous proliferation and circulating immune complexes were also evaluated. Whereas neutrophils from B51-positive subjects showed an increase in the chemotactic response toward casein (P = 0.003) and LPS (P = 0.033) and also in the PMA-induced superoxide production (P = 0.008) no evidence of enhanced lymphocyte activation emerged. These results suggest that the HLA region can exert a regulatory control on PMN functions.
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PMID:HLA B51 antigen associated with neutrophil hyper-reactivity. 182 11

Polyarteritis nodosa is a diffuse vasculitis of small- and medium-sized muscular arteries that can involve the vessels of one or several organ systems. We report an 11-year-old boy with severe atopic dermatitis further complicated with erythroderma and cerebellar symptoms. Laboratory studies showed negative antinuclear antibodies and antineutrophil cytoplasmic antibodies; increased serum complement, IgG cryoglobulins 96.8 mg/dL (0-80) and serum IgG 2,160 mg/dL (613-1,295), and positive alpha-cardiolipin test. The IgE value was high. Skin prick tests and RAST were positive for dust mites and casein. The viral profile was negative. Imaging studies disclosed tortuousity in the vessels and ischemic infarction in the cerebellum and thalamus. A diagnosis of polyarteritis nodosa with central nervous system involvement was made. The patient improved with immunosuppressive therapy and thalidomide. Currently, the patient is controlled and in a rehabilitation program. Other cases of polyarteritis nodosa associated with atopic dermatitis have not been reported.
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PMID:Central nervous system involvement in a child with polyarteritis nodosa and severe atopic dermatitis. 1256 51