Alpha 1-antitrypsin (alpha 1-AT) is the major inhibitor of proteinase 3 (PR3), the main target antigen of antineutrophil cytoplasm antibodies (ANCA) in Wegener's granulomatosis. alpha 1-AT is encoded by a polymorphic gene, with over 75 alleles, defining severely, medium and non-deficient protease inhibitor (PI) phenotypes. We describe the association of severely and medium deficient PI phenotypes with anti-PR3 positive systemic vasculitis, and postulate a pathogenetic role for alpha 1-AT deficiency and the occurrence of ANCA, with specificity for PR3 in a subgroup of patients with Wegener's granulomatosis.

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13 episodes of bacteremia caused by Pasteurella multocida were seen in a general hospital during a 12-year period. All the patients had an underlying disease (77% had cirrhosis) and 2 were receiving chemotherapy for hematologic malignancy. There was a numerical preponderance of male patients (69%). In 5/13 cases a recent animal-derived trauma could be found. In the other cases the source of the infecting organism was thought to be endogenous (from patients' own pharyngeal commensal flora) or secondary to contact with secretions of a pet animal. The clinical presentation of sepsis caused by this organism was nonspecific. Hypotension was seen in 5 cases. Localized sites of infection were certain in 6 and only clinically suspected in 4 other cases. The overall mortality rate was 31%. The administration of ampicillin seems the appropriate therapy for Pasteurella multocida bacteremia.

Our study suggests for the first time that inflammatory aortic involvement present at onset of GCA could predict a more chronic/relapsing course of GCA, with higher steroid requirements and an increased risk for vascular events in the long term.

Neisseria elongata subsp. nitroreducens, an usual commensal of the nasopharynx, can cause serious infections, usually endocarditis. A case of Neisseria elongata endocarditis complicating mitral valve prolapse in a patient allergic to penicillin is reported. Cure was obtained with a combination of ceftriaxone and gentamicin, there being no requirement for surgery during the acute phase of the disease.