Anaphylaxis: an analysis of cases evaluated at the Puerto Rico Medical Center over a ten-year period

OBJECTIVE: To examine the clinical characteristics of patients with anaphylactic reactions evaluated at the Puerto Rico Medical Center over a ten year period. BACKGROUND: Anaphylaxis, an immunologic reaction classically initiated by the combination of an antigen and a mast cell fixed antibody (usually IgE), still carries a fatality rate of 500 to 1000 cases per year in the United States. It constitutes a medical emergency that needs to be identified promptly in order to install appropriate treatment. No studies of this condition have been conducted in Puerto Rico, specifically to assess the clinical presentation, main causes and outcome. METHODS: Eighty-three records of patients with a diagnosis of anaphylaxis were screened by retrospective and concurrent analysis. Of these, only 51 fulfilled the diagnostic criteria of anaphylaxis. Specific data gathered from those records assessed the clinical characteristics of each case, precipitating factors, severity of the reaction and outcome. A standard form was used for data gathering. A grading system was utilized to classify the severity of the clinical episodes. RESULTS: Cutaneous features were the most commonly found manifestations of anaphylactic reactions in the studied group. Only reactions graded 2 and 3 were identified. Reactions to medications were the most frequent identifiable causes of the entity. Multiple sensitivities to different allergens were not predictive of this clinical condition. CONCLUSIONS: The identification in this study that only cases with the more severe grades of anaphylaxis were evaluated and treated at our center, the inability to recognize an inciting cause in about one third of the patient sample and the fact that a minority of the treated patients received subsequent follow-up by an allergist, reflect the need to promote the training of physicians in the field of allergy in Puerto Rico and the continued education of all physicians in the Island regarding this clinical disorder.

Rheumatologic manifestations in patients with selected primary immunodeficiencies evaluated at the University Hospital

OBJECTIVE: To characterize an IgA deficient and common variable immunodeficiency (CVI) group of patients in terms of the presence of rheumatologic manifestations. BACKGROUND: Although the molecular basis of some of the primary immunodeficiencies has been elucidated, it has not been possible to explain why in most cases these conditions are often associated with autoimmune manifestations, besides infections. The concomitant inability to fight infections adequately (immunodeficiency) and an inordinate reaction of the immune system to self components (autoimmunity) has been a perplexing situation. METHODS: The clinical and immunological profile of 71 patients fulfilling the diagnostic criteria of selective IgA deficiency (n=38) and common variable immunodeficiency (n=33) were evaluated for concurrent rheumatologic manifestations after a thorough medical history, physical examination and pertinent immunological parameters. RESULTS: The most common autoimmune conditions identified in patients with selective IgA deficiency were Crohn's disease and systemic lupus erythematosus (SLE); while immune thrombocytopenic purpura and Crohn's disease were the most common disorders associated to CVI. Anti-IgA antibodies were only found in 26.6(95C.I. 10.1-51.4) of patients with selective IgA deficiency but were present in all patients with that condition and SLE. Fifty per cent patients with CVI and ITP exhibited ANA positivity. CONCLUSIONS: The IgA-deficient group of patients in this study showed a higher prevalence of autoimmune conditions and greater positivity for ANA as compared to patients with CVI. In contrast to other reports with around 44positivity of anti-IgA antibodies in selective IgA patients these were only present in 263of patients with that disorder in this study. The high prevalence of antinuclear antibodies not associated with any clinical autoimmune condition in the IgA-deficient patients in this study will need to be further explored to ascertain why IgA-deficient patients may be at an increased risk of autoimmunity. Inflammatory bowel disease (Crohn's disease and ulcerative colitis) constituted the most common clinical autoimmune manifestations in both groups of patients studied. ITP was the commonest organ-specific autoimmune condition identified in the CVI group, as reported in previous publications. The limited number of patients studied does not allow a reliable estimate of the prevalence of SLE in the IgA-deficient population analyzed.