Fifteen premature newborns with hyaline membrane disease causing acute respiratory distress were evaluated for complement activation. A high intrapulmonary right-to-left shunt and marked arterial-alveolar oxygen difference indicated the severity of the respiratory failure. Twenty preterm healthy infants served as controls. Total haemolytic activity, plasma concentrations of complement components and regulatory proteins (C3, C4, C1-inhibitor, factors H and I) as well as activation products (C3a, C3dg, C1rsC1-inhibitor, C3b(Bb)P) gave no evidence of significant complement activation. Functional activity of the ubiquitous regulatory protein C1-inhibitor was significantly reduced without impact on classical pathway activation. These data suggest that, in contrast to the adult form of respiratory distress syndrome, the low-pressure pulmonary oedema characterising hyaline membrane disease is not mediated by activation of the complement system.

No abstract

Brazilian pemphigus foliaceus is an autoimmune blistering skin disease of man that has a very high incidence in a confined geographical distribution. Rocket immunoelectrophoresis of plasma showed increased levels of complement fragments, C4d and Ba, indicating activation of complement through both the classical and the alternative pathways. Less sensitive methods such as CH50, total C3 and C4 did not demonstrate this activation, and immune complex measurements were within normal range. While complement may not be absolutely necessary for the development of skin lesions, our longitudinal studies show that activation of complement is at its highest during the most active phase of the disease.

RESUMO -Encontramos 16 casos com vacúolos marginados entre 1400 biópsias musculares cujo diagnóstico final foi miosite com corpos de inclusão citoplasmática esporádica (MCIC) (4 casos), atrofia muscular espinhal juvenil (6 casos), miopatias distais (3 casos), distrofia das cinturas pélvica e escapular (2 casos) e neuropatia periférica (1 caso). Foram utilizados anticorpos monoclonais contra linfócitos T totais e subpopulações (CD4+ e CD8+), linfócitos B, macrófagos, células exterminadoras naturais (NK), imunoglobulinas e porção C3 do complemento. A análise foi quantitativa e de acordo com o local de acúmulo (interstício, intra-fibra e perivascular). Linfócitos CD8+ foram encontrados no interstício na maioria dos casos, sendo menos comuns dentro das fibras musculares e raros no espaço perivascular. Os casos de MCIC apresentaram maior número de linfócitos CD8+ se comparados às outras doenças. A proporção de células CD8+/CD4+ foi maior na MCIC do que nas outras doenças. Existiam macrófagos em grande proporção na MCIC, miopatias distais e em um dos casos de distrofia das cinturas pélvica e escapular. Células NK foram frequentes no interstício nos casos de MCIC e mais raras nas outras doenças. Houve maior depósito de imunoglobulinas e complemento nos casos de MCIC do que nas demais doenças. O grande número de células CD8+ e a relação CD8+/CD4+ podem auxiliar no diagnóstico diferencial da MCIC de outras doenças neuromusculares com vacúolos marginados. PALAVRAS-CHAVE: imunocitoquímica, vacúolos marginados, inclusão citoplasmática, miosite com corpos de inclusão citoplasmática, atrofia muscular espinhal juvenil, miopatias distais, distrofia muscular das cinturas pélvica e escapular. Immunocytochemical analysis of the inflammatory infiltrate in inclusion body myositis and other neuromuscular disorders with rimmed vacuolesABSTRACT -Among 1400 muscle biopsies, we found 16 cases with rimmed vacuoles whose diagnosis were sporadic inclusion body myositis (IBM) (4 cases), juvenile spinal muscular atrophy (6 cases), distal myopathies (3 cases), limb-girdle muscular dystrophy (2 cases), and peripheral neuropathy (1 case). Monoclonal antibodies reactive for T lymphocytes and subsets, B lymphocytes, macrophages, natural killer cells, immunoglobulins, and complement were used to analyze the inflammatory infiltrate. The analysis was quantitative and according to the site of accumulation (interstitial, endomysial, and perivascular). The immunocytochemical analysis showed CD8+ lymphocytes in the interstitial in most cases, occasionally inside of muscle fibers, and rarely in the perivascular region. The IBM cases had an increased number of CD8+ lymphocytes comparing with the other diseases. CD8+/CD4+ ratio was increased in IBM compared with the other diseases. Macrophages were frequent in IBM, distal myopathy, and one case of limb-girdle muscular dystrophy. Natural killer cells were frequent at interstitial Serviço de Doenças