Immune complexes and reticuloendothelial system function in human disease.

The interrelation between the presence of circulating antigen-antibody complexes and the functional status of reticuloendothelial system Fc-receptors was studied in patients with systemic lupus erythematosus and Sjogren's syndrome. Both groups of patients had a high prevalence of circulating immune complexes as detected by the 125I-Clq binding assay and the Raji cell radioimmune assay. A number of patients with both diseases were found to have abnormal reticuloendothelial system Fc-receptor function, as measured by the clearance of IgG-sensitized, 51Cr-labeled autologous erythrocytes.

Sjögren's syndrome (Sicca syndrome): current issues.

This paper outlines the clinical, serologic, and immunogenetic differences and similarities of Sjögren's syndrome alone (primary) and Sjögren's syndrome associated with rheumatoid arthritis and systemic lupus erythematosus (secondary). The immunoregulation in Sjögren's syndrome is discussed and the incidence of immune complex-like material, its nature, pathophysiology, and clearance by the Fc recptor of the reticuloendothelial system presented.

Small intestinal biopsies and HLA types in dermatitis herpetiformis patients with granular and linear IgA skin deposits.

In this study we determined whether dermatitis herpetiformis patients whose skin contained linear IgA deposits differ from those whose skin contained granular IgA deposits with regard to the presence of gluten-sensitive enteropathy and with regard to the prevalence of certain histocompatibility antigens. We performed multiple Rubin tube intestinal biopsies on 11 patients, 6 with linear and 5 with granular IgA deposits. The gut biopsies were evaluated histopathologically in a blinded fashion.

Necrolytic migratory erythema without glucagonoma.

Two patients with clinical and histologic findings consistent with necrolytic migratory erythema are presented. Unlike previously described patients with this disorder, neither patient had substantially elevated glucagon levels nor an associated pancreatic islet cell tumor. The cause of the skin disease in these patients remains unknown but may be related to the underlying small-bowel disorder present in both.

Sjögren's syndrome: a defect in reticuloendothelial system Fc-receptor-specific clearance.

To determine the functional status of reticuloendothelial system Fc receptors in patients with Sjögren's syndrome, we studied the rate of clearance from the circulation of 51Cr-labeled IgG-sensitized autologous erythrocytes in 19 patients. Fc-receptor-mediated clearance was abnormal in 12 of the 19 patients, with half-lives ranging from 80 to 356 min. There was a significant correlation between clearance rates and clinical manifestations of disease.

Circulating immune complexes in acute schistosomiasis.

The sera of patients with acute and chronic schistosomasis were tested for the presence of circulating immune complexes with the 125I-Clq binding assay. Fourteen out of fifteen (93%) patients with acute schistosomiasis had elevated 125I-Clq binding activity, while only two out of eleven (18%) patients with chronic disease had C1q binding complexes. This difference was significant (P less than 0.

Immunologic reactivity in the hypereosinophilic syndrome.

Because previous studies have suggested an important link between eosinophilia and immunologic reactivity, we investigated various components of the immune system in a large number of patients with the idiopathic hypereosinophilic syndrome (HES) to elucidate a possible role for immunologic phenomena in the etiology and pathogenesis of this disease. Immunoglobulin G, A, or M levels were only rarely abnormal. However, in 8 of 21 (38%) patients with HES, IgE levels were markedly elevated suggesting an association of an IgE-mediated mechanism with eosinophilia in this subgroup.

Pruritic urticarial papules and plaques of pregnancy.

We report an intensely pruritic cutaneous eruption that occurs in the third trimester of pregnancy. The clinical manifestations include erythematous urticarial papules and plaques that begin on the abdomen and spread to involve the thighs and occasionally the buttocks and arms. This dermatosis appears to be clinically distinct from previously described pruritic eruptions occurring in pregnancy.

Defective reticuloendothelial system Fc-receptor function in systemic lupus erythematosus.

To determine whether reticuloendothelial-system immunospecific Fc-receptor function is abnormal in patients with systemic lupus erythematosus, we studied the clearance of IgG-sensitized 51Cr-labeled erythrocytes by these splenic macrophage membrane receptors in 15 untreated patients. Fc-specific clearance rates were strikingly abnormal in 13 of 15 patients (half-times ranging from 80 to 2256 minutes, P less than 0.001 as compared to controls).

Antibodies to type II collagen in relapsing polychondritis.

Relapsing polychondritis is a disorder of unknown cause characterized by the destruction of cartilage. To test the hypothesis that immunologic mechanisms are involved in the pathogenesis of relapsing polychondritis, we analyzed the serum of 15 patients for the presence of antibodies to cartilage. Antibodies to Type II (cartilage) collagen were found in the serum of five patients at the time of acute symptoms.

Receptor specific clearance by the reticuloendothelial system in chronic liver diseases. Demonstration of defective C3b-specific clearance in primary biliary cirrhosis.

An approach to the assessment of reticuloendothelial function that quantitates clearance specifically mediated by membrane receptors for C3b and immunoglobulin (Ig)G has been applied in man. Clearance of isologous erythrocytes coated with IgM or C3b or coated with IgG were examined in patients with primary biliary cirrhosis (PBC), chronic hepatitis, or alcoholic cirrhosis and normal control subjects and compared with the clearance of aggregated human serum albumin. Clearance of these three types of particles varied independently.

Evaluation of the adverse effects of long-term hyposensitization.

This study was undertaken to determine if long-term hyposensitization causes late sequelae, particularly those reflecting aberrant immunologic responses. Atopic individuals receiving five or more years of hyposensitization with allergenic extracts showed no increased autoimmune, collagen vascular, or lymphoproliferative disease. In addition, chronic hyposensitization did not have adverse effects on immunologic reactivity as assessed by a number of immune parameters.

Immune complex glomerulonephritis in sicca syndrome.

In three patients with the sicca syndrome (Sjögren's syndrome), who were followed for one to seven years, glomerulonephritis developed. None of these patients fulfilled the diagnostic criteria for systemic lupus erythematosus. All of these patients had circulating immune complexes as detected by the Clq binding assay.

Fetal and maternal risk factors in herpes gestationis.

Forty-one cases of immunologically proved herpes gestationis (HG) are reviewed and there appears to be an increased risk of fetal morbidity and mortality. The onset of HG is most frequent in the second and third trimesters of pregnancy although postpartum onset or exacerbation is common. The presence of high-titer, antibasement membrane zone antibody seems to correlate with a severe clinical disease.

Scleroderma, Sjögren-like syndrome, and chronic graft-versus-host disease.

A patient with acute myelogenous leukemia treated with an allogeneic bone marrow transplant developed acute graft-versus-host disease manifested by severe diarrhea, hepatitis, and a cutaneous eruption. As the graft-versus-host disease progressed to the chronic phase, the patient developed marked cutaneous sclerosis and symptoms of xerophthalmia and xerostomia. Biopsy of his indurated skin showed features of both graft-versus-host disease and scleroderma.

Erythema elevatum diutinum: skin and systemic manifestations, immunologic studies, and successful treatment with dapsone.

Erythema elevatum diutinum is a disease characterized by red, pink, purple and yellow cutaneous papules, nodules, and plaques distributed mainly over extensor surfaces. Histologically, there is leukocytoclastic vasculitis with fibrinoid necrosis of the upper and mid dermal vessel walls. Two of the five patients presented had moderately severe arthralgias and, unlike previously reported cases, three of them had a long history of bacterial infections and one had a coincident IgA monoclonal gammopathy.

Effect of sulfones on complement deposition in dermatitis herpetiformis and on complement-mediated guinea-pig reactions.

The role of complement and the mechanism of sulfone action in dermatitis herpetiformis (DH) have not yet been established; prior studies have presented conflicting data regarding the effect of sulfones on complement activation and deposition. Thirty-eight DH patients were studied. Twenty-four of 25 perilesional skin biopsies and 50 of 67 normal-appearing skin biopsies showed the third component of complement (C3) deposited in areas corresponding to those of IgA deposition.