HL-A antigens in Behcet's disease.

Twenty-six patients with Behcet's disease had complete HL-A typing. No patient had an HL-A antigen in significant excess. No patient had an HL-A antigen in significant excess. Only two patients had the B27 antigen: both patients had uveitis, but eight others who also had uveitis were B27 negative. No patient had ankylosing spondylitis or sacroilitis. Behcet's disease, although bearing some clinical resemblances to diseases related to the B27 antigen, i.e. ankylosing spondylitis and Reiter's syndrome, differs from them through the absence of spondylitis and because the association with B27, if it exists, is far weaker than that in ankylosing spondylitis or Reiter's disease.     

HL-A antigens in Takayasu's disease.

Takayasu's disease is characterized by a "pulseless" condition which most often occurs in young females from Asian or South American areas. The cause of this disease remains obscure. Recently, we encountered monozygotic, Japanese, identical twin sisters, both of whom were diagnosed as having Takayasu's disease. The parents, two sisters, and one brother are healthy. HL-A typing analyses revealed that one haplotype found in the father had passed only to these twins. Such observations led us to search HL-A typing in Takayasu's disease to determine the possible participation of genetic factors in the pathogenesis of this morbid condition. Ten families, including that of our own patient, have been reported in the literature in Japan, as family cases of Takayasu's disease. HL-A typings in A and B locus analyzed in all family members of six families in attempts to find a common haplotype composed of A9, A10, B5, or BW40 in patients with Takayasu's disease, were confirmed statistically (chi 2 = 7.8, 0.01 less than p less than 0.05). In a population study, HL-A typing analyses of 65 patients with Takayasu's disease also revealed a high frequency of HL-A A10 and HL-A B5 with the level of 15.3 and 17.0 in the chi 2-test (p less than 10(-4)), as compared with the frequency in 128 healthy Japanese. These data strongly suggest that a genetic-related factor has to be given serious consideration.     

Immunological and chemical purity of papain-solubilized HL-A antigens.

Three preparations of purified papain-solublized HL-A antigens have been radiolabeled by reductive methylation using formaldehyde and potassium boro[3H]hydride, and their reaction with specific HL-A antisera has been investigated. Greater than 99 percent of the radioactivity in the [3H]HL-A2 preparation could be complexed with several HL-A2 antisera, but not with specificity controls. The other two preparations, which contained mixtures of HL-A antigenic specificities (HL-A7,12 an HL-A3,W25;12,27), showed 63 per cent and 70 per cent complex formation with mixtures of the appropriate HL-A antisera. The N-terminal amino acid of both subunits has been determined for the three HL-A antigen preparations. In all cases the only detectable N-terminal amino acids were isoleucine for the small subunits, beta-2-microblogulin, and glycine for the larger subunit.     

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.Glomerular histology by light and electron microscopy revealed changes compatible with membranoproliferative glomerulonephritis in two of the patients and membranous glomerulonephritis in the third.All patients showed rapid improvement in renal function following moderate doses of corticosteroids. In addition, the treatment decreased the level of circulating immune complexes in two patients who were followed for a sufficient period of time.     

HL-A and carpal tunnel syndrome

Summary One hundred and five patients suffering from carpal tunnel syndrome were HL-A typed. No linkage could be demonstrated between HL-A phenotypes and this syndrome.     

Increased risk of lymphoma in sicca syndrome.

The risk of cancer was ascertained in 136 women with sicca syndrome followed at the National Institutes of Health (NIH). Seven patients developed non-Hodgkin's lymphoma from 6 months to 13 years after their first admission to NIH. This was 43.8 times (P less than 0.01) the incidence expected from the rates of cancer prevailing among women of the same age range in the general population during this time. In addition, three cases of Waldenstr?m's macroglobulinemia occurred in this study group. Eight patients developed cancers other than lymphoma, similar to the number expected based on the rates prevailing in the general population. Patients with a history of parotid enlargement, splenomegaly, and lymphadenopahy had an increased risk of lymphoma. These clinical conditions did not appear to be early manifestations of undiagnosed lymphoma but rather seemed to identify a subgroup of patients with sicca syndrome with marked lymphoid reactivity, who had a particularly high risk of subsequently developing lymphoma.     

Matching of histocompatibility (HL-A) antigens for platelet transfusion.

The average in vivo platelet survival was measured by both per cent recoveries (%R) and half-lives (t 1/2) of 51Cr-labeled platelets in 69 transfusions given to 43 thrombacytopenic patients. The results demonstrated that (1) increase in the number of HL-A incompatibilities in the platelet donor was significantly associated with decreased %R and t 1/2 of the infused platelets; (2) survival of transfused platelets from HL-A-matched single donors was consistently superior to those pooled from several grossly mismatched donors; and (3) survival of platelets infused when patients had circulating lymphocytotoxic antibodies was consistently lower than when patients did not have such antibodies, regardless of whether it was the first, second, or third such infusion.     

Salivary scanning in rheumatoid arthritis with sicca syndrome.

Forty-nine patients were studied prospectively by salivary scanning to assess the value of this investigation in possible Sjögren''s syndrome (SS). Twenty-three had rheumatoid arthritis (RA) with sicca symptoms and a positive Schirmer''s test. Fifteen had RA with no sicca symptoms and a negative Schirmer''s test. Eleven had osteoarthrosis (OA) with no sicca symptoms and a negative Schirmer''s test. Scanning differentiated only poorly between the three groups. We conclude that it has only limited application in the diagnosis of SS associated with RA.     

Studies on new HL-A antigens of the first segregant series

Abstract. Population and family studies are reported showing that W 19 (equivalent designations: Li, LA-W, Thompson, AO 28) belonging to the first segregant series, is heterogeneous and is probably composed of at least two and possibly four different specificities. One of them, designated as GE 33, is defined by two sera from multiparous women; another AO 28 is defined by serum THA from Amos and serum Thompson from Walford. Two additional specificities provisionally designated as LC 26.1 and LC 26.2 are less clearly defined by multispecific sera included in the last Histocompatibility Workshop. The existence of cross-reactions between different HL-A antigens of the same segregant series considerably complicates the accurate definition of new antigens. Other already known HL-A antigens will probably have to be split into different ‘parts’, further increasing the already considerable polymorphism of the HL-A system.     

Change in the incidence of HL-A antigens in diabetes mellitus

Changes in the incidence of the HLA system antigens (A1, A2, A3, A9, A10, A11, A19, A28, A29, B5, B7, B8, B13, B14, B15, B16, B17, B18, B21, B22, B27, B35, B37, B40, B41) was studied in 1134 healthy persons and in 147 patients with diabetes mellitus. In comparison with healthy persons, patients with juvenile diabetes mellitus (62) displayed a significant increase in the incidence of antigens B8, B15, B35, and A10, and patients with adult diabetes mellitus with normal weight (35)--of antigen B8. When adult diabetes mellitus was accompanied by obesity (50) a significant rise in the occurrence of antigen A10 was revealed.     

HL-A antigens in Europeans and Maoris with rheumatic fever and rheumatic heart disease.

Using a standard microtoxicity technique of tissue typing, the distribution of tissue antigens in 75 Maoris and 514 European disease-free blood donors was determined. Fifty Maori and 50 Europeans with rheumatic fever or rheumatic heart disease were compared with each control group. Normal Maoris had HL-A3 less frequently than Europeans (P less than .0005). HL-A28 was reduced (P less than .005) and HL-A17 increased in European patients (P less than .0005). In Maori patients there were minor differences in the frequency of HL-A3 and 8, which were increased, and HL-A10, which was diminished.     

Angioimmunoblastic lymphadenopathy with dysproteinaemia (AILD) and sicca syndrome.

We report a case of AILD and sicca syndrome. The patient had presented with renal insufficiency, lymphadenopathy, hepatosplenomegaly, polyclonal hypergammaglobulinaemia, dryness of the eyes and mouth. Lip biopsy specimens showed an unusual cellular infiltrate similar to his kidney lesions. Data from the eight previously reported cases support the hypothesis that the association is a distinct pathological entity differing from pseudolymphoma and malignant lymphoma, which occur in the course of Sjögren''s syndrome. The recognition of AILD is important because lymphoproliferation may lead to death after a few months.