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K B Wiesner

Publications and source records attributed to K B Wiesner.

10 recordsLinked to original sources

The immune complex pathogenesis of glomerulonephritis and pulmonary vasculitis in Behçet's disease.

A kidney and lung biopsy were performed on a patient with active Behçet's disease with renal and pulmonary involvement. Histologic, immunohistochemical and electron microscopic studies of the kidney biopsy specimen revealed a focal segmental necrotizing glomerulonephritis characterized by the presence of numerous subendothelial and occasional intramembranous deposits containing immunoglobulin G (IgG), the third component of complement (C3), the fourth component of complement (C4) and fibrin(ogen). Histologic and immunohistochemical studies of the lung biopsy specimen showed an acute venulitis and septal capillaritis associated with the presence of identical deposits within the walls of affected vessels. Circulating immune complexes were detected in the patient's serum by Raji cell assay. The findings indicate that the glomerulonephritis and pulmonary vasculitis occasionally occurring in Behçet's disease are due to the deposition of circulating antigen-antibody complexes. In addition, they strongly suggest that the majority of the major and minor manifestations of the disease, such as uveitis, cutaneous vasculitis, synovitis and meningoencephalitis, are a result of vascular immune complex deposition.

Behcet Syndrome

Roentgenologic, immunologic, and therapeutic study of erosive (inflammatory) osteoarthritis.

The terms erosive or inflammatory osteoarthritis refer to an inflammatory condition of the interphalangeal joints of the hand. In this report, observations of 15 patients with erosive osteoarthritis are described. The principal clinical features are symmetrical synovitis of the interphalangeal joints of the hand, the knees, and the first carpometacarpal, interphalangeal, and metacarpophalangeal joints. The principal roentgenographic features are productive and destructive osseous changes. These changes, found particularly at the interphalangeal joints of the hand, consist of both central and peripheral articular erosions and cysts associated with adjacent osteophyte formation. Serologic abnormalities commonly found in rheumatoid arthritis are rarely encountered. In two thirds of the patients, the synovial fluid is noninflammatory; in the remainder, it is mildly to severely inflammatory. The injection of intra-articular corticosteroids predictably results in decreased synovitis but does not seem to affect the subsequent development of erosions.

Aged

Immunopathogenesis of Libman-Sacks endocarditis. Assessment by light and immunofluorescent microscopy in two patients.

The possible contribution of immunological mechanisms in the development of Libman-Sacks endocarditis was studied in 2 patients with systemic lupus erythematosus who underwent aortic valve replacement. Sections of verrucous lesions, stained with haematoxylin and eosin, showed three apparently distinct zones: an outer exudative zone of fibrin, nuclear debris, and haematoxylin-stained bodies; a middle organizing zone of proliferating capillaries and fibroblasts; and an inner zone of neovascularization which showed distinct, thin-walled junctional vessels. The striking finding was the apparently selective deposition of immunoglobulins and complement identified by direct immunofluorescence, within the walls of the small junctional vessels of the zone of neovascularization. We suggest that the observed immune deposits are immune complexes and that circulating immune complexes may play a critical role in the growth and proliferation of the verrucous lesion.

Aortic Valve

Clinical, radiographic and pathologic abnormalities in calcium pyrophosphate dihydrate deposition disease (CPPD): pseudogout.

Clinical, radiographic and pathologic abnormalities in calcium pyrophosphate dihydrate deposition disease (CPPD) (pseudogout) are outlined in an investigation of 85 patients with definite or probable disease and available cadaveric and human surgical material. Pyrophosphate arthropathy produced distinctive roentgenographic abnormalities with were most frequent in the knee, wrist and metacarpophalangeal joints. Although the alterations superficially resembled osteoarthritis, they were frequently more severe and progressive with extensive fragmentation of bone, causing intra-articular osseous bodies. Pyrophosphate arthropathy occurred in unusual locations, such as the radiocarpal compartment of the wrist, elbow, and patellofemoral compartment of the knee. These characteristics allow the radiologist to suggest a probable diagnosis of CPPD even in the absence of articular calcification.

Aged

HLA-B27 and modified bone formation.

Of the many associations between histocompatibility antigens and human diseases a prominent one is that between HLA-B27 and inflammatory arthropathies. Hypotheses to explain this association include the B27 gene being linked to a specific immune-response gene required for disease expression and the B27 antigen acting via molecular mimicry with a microorganism or as a microorganism receptor. Alternatively, the HLA-B27 gene might be closely related to a gene which influences bone formation. The finding of a significant association between B27 and Forestier's disease, a disease characterised by abundant new bone formation, supports such a hypothesis.

Aged

The association of HL-A B27 with Forestier's disease (vertebral ankylosing hyperostosis).

Despite the lack of apophyseal or sacroiliac joint involvement, Forestier's disease (vertebral ankylosing hyperostosis) shares with the inflammatory forms of spondylitis, the roentgenographic appearance of spinal new bone formation. Because of this apparent similarity, the prevalence of the HL-A B27 antigen was determined in 47 white patients with Forestier's disease. Sixteen of the patients (34 per cent) possessed the B27 antigen (P is less than 0.001). The mere presence of B27 therefore, does not confirm the diagnosis of inflammatory spondylitis in all patients with roentgenographic evidence of osseous bridging. Applying available epidemiologic information, these data further suggest that patients with the B27 antigen may be at substantial risk of developing Forestier's disease. As Forestier's disease and virtually all of the other B27 associated arthropathies manifest abundant new bone, an association may exist between this antigen and genes controlling new bone formation.

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Radiographic abnormalities of rheumatoid arthritis in patients with diffuse idiopathic skeletal hyperostosis.

Radiographic abnormalities of rheumatoid arthritis (RA) in 8 patients with diffuse idiopathic skeletal hyperostosis (DISH) included atypical features: lack of osteoporosis, bone sclerosis and proliferation about erosions, osteophytosis, and bony ankylosis. Atypical clinical features included a high incidence of flexion contractures of elbows, wrists, ankles, or knees. It is not surprising that bone production occurs about involved articulations in patients with RA-DISH, as the latter disorder is characterized by bony proliferation at sites of ligament and tendon attachment to bone in the axial and extraaxial skeleton, perhaps related to stress.

Aged