[The effect of clinical and animal experimental conditions on autogenous defense].
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Membranoproliferative (mesangio-capillary) glomerulonephritis (MPGN) has been divided into two types: type 1, MPGN with subendothelial deposits; type 2, MPGN with intramembranous dense deposits. Although the types are clinically similar, the light, immunofluorescent, and electron microscopical data differ. The cause in the majority of the cases is unknown, but chronic antigenemia as a pathogenic mechanism is suggested in cases associated with chronic bacteremia, chronic hepatitis, parasitic infections, certain blood dyscrasias, and partiallipodystrophy. Both the classic and the alternate pathway of complement activation may be important. The mechanism of the formation of the dense intramembranous deposits is unknown, but activation of the alternate pathway of complement appears to be an important factor. It is emphasized that while there are two basic pathologic types of MPGN, there are diverse clinical syndromes associated with these lesions, the recognition of which is crucial.
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The complement system plays an important role in defence of the host against infection and in the clearance of immune complexes. Defects in complement proteins are often associated with infections or auto/immune complex diseases. Investigation of complement is useful for diagnosis and following of auto-immune diseases. The aim of this Article is to provide an overview of important applications of complement in medicine, emphasizing the role of complement in pathogenesis and the usefulness of measurements of complement proteins in diagnosis and assessment of the evolution of disease states. Emphasis has been placed on practical applications and understanding basic mechanisms of disease. The best screen for complement deficiencies or significant activation is the CH50, which measures total classical pathway activity and the measurement of C3 and C4. The absence or decrease of multiple components is usually due to consumption of complement. Complete lack of CH50 associated with normal C3 antigen is a strong indication for complement deficiency and should be followed up with further tests to determine which component is missing.
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A patient with systemic lupus erythematosus (SLE) and lupus retinopathy showed resolution of subretinal edema documented with fluorescein angiography. Subsequently at autopsy, immunofluorescence studies disclosed ocular deposition of immunoglobulins in the vascular layer of choroid capillaries and basement membranes of ciliary processes and bulbar conjunctivas. To our knowledge, these findings represent the first reported documentation of probable immune-complex ocular vasculitis in lupus retinopathy using immunofluorescent techniques, and they support the hypothesis that lupus retinopathy is caused by immune complex deposition as are other manifestations of SLE.
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