The clinical spectrum of antinuclear antibodies associated with the nuclear dense fine speckled immunofluorescence pattern.

OBJECTIVE: Autoantibodies to lens epithelium-derived growth factor (LEDGF) depict a distinctive nuclear dense fine speckled (DFS) pattern in the indirect immunofluorescence antinuclear antibody assay (IIF-ANA). Definition of the clinical spectrum associated with anti-LEDGF antibodies has been evolving over the last decade. We investigated the frequency, clinical spectrum, and immunologic specificity of the DFS pattern in a general clinical laboratory routine. METHODS. All serum samples entered for IIF-ANA determination within a 2 year period were examined for the DFS pattern. Positive samples with consistent clinical information were studied further by IIF with isotype-specific conjugate and immunoblot analysis. RESULTS: Among 13,641 ANA-positive samples, 5081 (37%) presented the DFS pattern. Within a 6 month nested period, there were 650 samples with DFS pattern, and consistent clinical data were available for 81 of these. DFS reactivity was mainly due to IgG. Most samples (86%) presented titer > or = 1/640. Eighty of the 81 DFS samples reacted with a 75 kDa band that comigrated with the band elicited by the standard anti-LEDGF serum. Antibodies that were affinity-purified from the 75 kDa band reproduced the DFS pattern on IIF-ANA. The clinical spectrum associated with DFS reactivity included autoimmune diseases (39%) and an array of nonautoimmune conditions (61%). Among the autoimmune patients, over half presented evidence of autoimmune thyroiditis. CONCLUSION: Anti-LEDGF/p75 antibodies are a common finding among ANA-positive individuals with no evidence of rheumatic autoimmune disease, and should be regarded as a low specificity finding even when in moderate or high titer.

Immunoglobulin M (IgM)-glycoinositolphospholipid enzyme-linked immunosorbent assay: an immunoenzymatic assay for discrimination between patients with acute toxoplasmosis and those with persistent parasite-specific IgM antibodies.

In the present study we developed an enzyme-linked immunosorbent assay (ELISA) to measure immunoglobulin M (IgM) specific for glycoinositolphospholipids (GIPL) derived from tachyzoite membrane (IgM-GIPL ELISA). The sensitivity and specificity of the assay were compared with those of commercially available Toxoplasma-specific IgM serological tests, namely, immunofluorescence assay (IFA) with fixed tachyzoites and capture ELISA employing tachyzoite extracts. Our results show that all patients with acute toxoplasmosis, as determined by clinical data and conventional serological tests, were also positive by the IgM-GIPL ELISA. Interestingly, many patients that were classified as indeterminate, who had IgG with high avidity but positive results in the IgM-specific IFA and capture ELISA, were negative by the IgM-GIPL ELISA. Finally, we tested the sera from patients with rheumatoid arthritis and various parasitic infections and found no evidence of false positives in the IgM-GIPL ELISA.

Activity of bristles from an Amazonian lepidoptera, "Premolis semirufa", on the human complement system

O lepidóptero Premolis semirufa habita os seringais da regiao Amazônica. Os seringueiros denominam pararama a forma larval do inseto e pararamose a doença ocupacional causada pelo contato acidental com cerdas da larva ou casulo durante a coleta do látex. Os sintomas imediatos que se seguem ao contato sao locais e consistem em prurido intenso, edema e dor, que sao geralmente transitórios e desaparecem em poucos dias. Entretanto, em algumas circunstâncias, o processo torna-se crônico, podendo evoluir para a deformidade e imobilizaçao da articulaçao afetada, lembrando, sob alguns aspectos, artrite reumatóide. O estudo da açao in vitro de cerdas e substâncias solúveis associadas sobre o sistema complemento humano mostrou que tanto cerdas quanto extrato salino inativam complemento total e o componente C2, geram C3d a partir de C3, sem afetar os níveis de Clq. Estes resultados sugerem que a via clássica de ativaçao do complemento nao está envolvida no processo. A inativaçao direta de C2 e a geraçao de C3d sao compatíveis com a presença de substâncias solúveis com açao lítica associadas às cerdas, embora nao tenha sido excluída ativaçao paralela da via alternativa como mecanismo de produçao de C3d

Cartilage-hair hypoplasia syndrome: immunological evaluation of two cases

PURPOSE--Immunological evaluation of patients with cartilage-hair hypoplasia. TYPE--Prospective and retrospective studies. PLACE--Division of Allergy, Clinical Immunology and Rheumatology-Dept. of Pediatrics-"Escola Paulista de Medicina". PATIENTS--Two children with cartilage-hair hypoplasia syndrome. METHODS--Clinical and immunological evaluation. Humoral immunity (immunoglobulin levels, poliovirus antibodies, etc.) and T cell immunity (in vitro cultured lymphocytes stimulated with PHA, Con A and PWN, total T cell and subset determination) were studied. RESULTS--Cellular immunodeficiency and hypogammaglobulinemia were observed in one patient and normal values in the other. CONCLUSIONS--Immunological evaluation (cellular and humoral) should be performed in all patients with cartilage-hair hypoplasia.

Common variable immunodeficiency: a clinical and laboratory evaluation of l5 cases

The clinical and laboratory data for 15 patients with common variable immunodeficiency (CVI) (5 females and 10 males aged 3 years and 6 months to 40 years at first examination) were evaluated. The age of onset of infectious signs and symptoms ranged from 6 months to 35 years. Recurrent pulmonary infections predominated (86.6%), followed by chronic diarrhea (46.6%). Aproximately 60% of the patients with pulmonary complaints presented chronic sequelae (bronchiectasis). Two developed a polymyositis-like picture. No neoplasms were observed. All patients presented immunoglobulin levels below 300mg/dl and absence of globulin levels below 300mg/dl and absence of antibody responses to poliovirus and to hemagglutinin. Two patients were negative when tested for autoimmunity. Cell immunity tested by the limphoproliferative response in the presence of phytohemagglutinin was normal in 11 patients and depressed in 4. A decrease in the helper T population and inversion of the OKT4/8 ratio occured in 13. Cimetidine treatment (1200mg/day) applied to 5 patients for 4 weeks did not produce any clinical or laboratory improvement. Gamma globulin is the treatment of choice for these patients

Tratamento imunomodulador com cimetidina em pacientes com imunodeficiência comum variável / Immunomodulator treatment with cimetidine in patients with common variable immunodeficiency

A imunodeficiência comum variável (ICV) caracteriza-se clinicamente por infecçöes respiratórias de repetiçäo e níveis séricos de imunoglobulinas muito baixos ou ausentes. Nestes pacientes, observa-se também um aumento da populaçäo de células T supressoras (OKT8), com inversäo da relaçäo OKT4/OKT8. Como as células T supressoras possuem um receptor para H2, na tentativa de modular esta subpopulaçäo, têm sido usados antagonistas de H2, objetivando bloquear a atividade supressora e conseqüente produçäo de imunoglobulinas. em três pacientes com ICV tratados com cimetidina por quatro a oito semanas, näo observamos efeito benéfico desta droga em relaçäo ao quadro clínico, embora algumas alteraçöes in vitro tenham sido observadas

Avaliaçäo imunológica in vitro em crianças portadoras de asma brônquica grave / Evaluation immunologic in vitro in children carrier of disease bronchial asthma

Vários estudos tem relacionado a hiper IgE observada em pacientes atópicos à diminuiçäo da populaçäo de células T supressoras. Estudamos 27 crianças atópicas, com idades de 2 a 7m a 14 anos, com asma brônquica perene e grave. Sangue periférico foi colhido após 1 dia sem qualquer medicaçäo broncodilatadora e 7 dias sem corticosteróides. A dosagem de IgG, IgM e IgA foi feita por imunodifusäo radial e a de IgE por radioimunesaio. O número de linfócitos T totais, T supressores e T auxiliadores foi determinado utilizando-se anticorpos monocolonais (Ortho, OKT3, OKT4, OKT8). As culturas de linfócitos foram estimuladas com fitohemaglutinina (PHA) e os resultados expressos como índice de estimulaçäo. Nossos achados comparados com nossos valores normais mostraram: IgG normal, IgA baixa, hiper IgM e hiper IgE (padräo habitual observado em nossa populaçäo asmática). Os porcentuais de células OKT3, OKT4 e OKT8 bem como relaçäo OKT 4/8 estiveram dentro da normalidade. As culturas de linfócitos com plasma autólogo e homólogo se mostraram normais. Baseado nesses achados, concluímos que näo há alteraçöes imunológicas importantes em crianças com asma brônquica atópica tornando desnecessário o emprego de agentes imunomoduladores no tratamento da asma brônquica na criança