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PubMed1995

Systemic lupus erythematosus in Saudi patients.

Eighty-seven patients with systemic lupus erythematosus (SLE) were retrospectively studied in King Khalid University Hospital, Riyadh. There were 78 females and 9 males (F:M ratio of 9:1). The mean age (+/- SD) at onset and at diagnosis were 25.3 +/- 10.5 and 28.5 +/- 10.9 years, respectively, with peak incidence in the 20-30 year age group. Musculoskeletal (91%), constitutional (76%), cutaneous (72%) and renal (63%) manifestations occurred most frequently, while neuropsychiatric manifestations (26%), photosensitivity (26%) and oral ulcers (16%) were relatively less frequent. The most common laboratory abnormalities included ANA (98%), anti-DNA (93%), LE cells (66%) and lymphopenia (70%). There were seven deaths during the study period and most of them were related to renal failure and complications from infections. Overall, the pattern of SLE observed in the series was comparable to that observed in other series among Caucasians.

Adolescent↗
PubMed1981

Serologic studies in a family with heterozygous C2 deficiency.

Twelve family members of a patient with systemic lupus erythematosus (SLE) and heterozygous deficiency of the second component of complement (C2) were studied. Histocompatibility (HLA) typing was determined for A, B, and DR and MB antigens. Serum samples were tested for a variety of antinuclear antibodies (ANA), lymphocytotoxic antibodies and rheumatoid factors, and C2 levels were determined by hemolytic titration. Inheritance of C2D, the gene coding for C2, was limited to the haplotype HLA-A25, B18, DR2. Low but significant titers of ANA, rheumatoid arthritis nuclear antigen (RANA) and/or rheumatoid factors were found in eight of the nine adult family members without association with HLA haplotype. The sister of the proband had persistently strongly positive LE cell preparations for more than a decade and had joint pains while taking sulfa drugs. The son of the proband had leukemia. All other family members were healthy. We conclude that the increased incidence of rheumatic disease in persons with C2D deficiency is multifactorial and requires environmental factors or other hereditary factors unrelated to the HLA-A25, B18, DR2 haplotype. The C2D gene is clearly not associated with positive ANA tests or immunoprecipitins to RANA.

Adult↗
PubMed1987

The laboratory identification of serum antinuclear antibody in the dog.

An indirect immunofluorescence test with sections of rat liver as a substrate, proved useful in detecting antinuclear antibodies (ANA) in the dog. A specific anti-canine IgG reagent conjugated with fluorescein isothiocyanate was used. A proportion of normal dogs gave positive reactions at low titres. The presence of ANA was one of the criteria used to diagnose canine systemic lupus erythematosus. Some dogs within a general hospital population were also positive for the auto-antibody. The indirect immunofluorescence test with Trypanosoma brucei as a substrate was insensitive. The LE-cell test was laborious and insensitive. A commercial latex slide agglutination test used for detecting ANA in man gave false positive and false negative results in the dog. A commercial radioimmunoassay used in man gave many false positive results. A similar assay with synthetic DNA gave encouraging results and is worthy of further study.

Animals↗
PubMed1981

Antimalarials.

The antimalarials, chloroquine, hydroxychloroquine, and quinacrine, are used primarily for malaria; but they can be beneficial for cutaneous lupus erythematosus (LE), polymorphous light eruption, solar urticaria, and porphyria cutanea tarda. Antimalarials bind to deoxyribonucleic acid (DNA) which prevents DNA and ribonucleic acid (RNA) polymerase reactions and DNA heat inactivation; and they inhibit the LE cell phenomenon, antinuclear antibody reactions, and suppress lymphocyte transformation. By competing with calcium ion, they stabilize membranes and have an anesthetic effect. Their anti-inflammatory potential is due to their inhibition of hydrolytic enzymes, stabilization of lysosomes, interference with prostaglandin synthesis, blocking of chemotaxis, and antagonism of histamine responses. The antimalarials have no sunscreening properties. The most common toxic effects are cutaneous pigmentation, nausea, vomiting, diarrhea, mild ileus, and cycloplegia. There has been a reluctance to use chloroquine and hydroxychloroquine because of the possibility of retinopathy. However, if the "safe" daily dose limit of chloroquine, 2 mg per pound of body weight, and of hydroxychloroquine, 3.5 mg per pound of body weight, is followed, the chance of retinopathy is slight. Quinacrine does not cause retinopathy, but it has more cutaneous side effects than the other two agents.

Antimalarials↗
PubMed1982

Systemic lupus erythematosus in hereditary deficiency of the fourth component of complement.

Three patients from two families with complete hereditary deficiency of the fourth component of complement (C4) and systemic lupus erythematosus are described. The syndrome presented by these patients is characterized by early onset in life; exquisite sensitivity to sunlight and to cold exposure, the latter resulting Raynaud's phenomenon; and skin lesions involving not only exposed areas of the body but also palms and soles and presenting as butterfly rashes, maculopapular eruptions, and lesions similar to those of chronic discoid lupus erythematosus, with marked scaling, atrophy, and scarring. Lupus erythematosus (LE) cell tests were negative and antinuclear antibody (ANA) titers low or negative. The male patient of our series died at the age of 31/2 years from septicemia, whereas the two girls, aged 18 and 11 years, respectively, were alive at the time of writing. The C4-deficient gene is associated with HLA-Aw32, Bw38, and Bf S in one family and with HLA-A30, B18, DR7, and Bf S1 in the other family; the latter is the second family in which this HLA haplotype has been found to be associated with hereditary C4 deficiency.

Adolescent↗
PubMed1982

Diet-induced systemic lupus erythematosus (SLE) in primates.

Ten adult, female cynomolgus macaques were randomly assigned to two equal groups: (1) semipurified diet (SPD); and (2) SPD with 45% ground alfalfa seed (AS). Both groups were studied at monthly intervals after 5 mo on their respective diets. Control animals had a mean hematocrit (Hct) of 43 +/- 2%, negative antiglobulin (AG), antinuclear antibody (ANA) and LE cell tests. Mean values for C3 and C4 were 309 +/- 47 mg/dl and 35 +/- 7 mg/dl, respectively. Mean serum binding to radiolabeled double stranded deoxyribonucleic acid (dsDNA) was 1.9 +/- 0.2%. Three of five animals fed AS developed signs of an SLE-like illness characterized by AG-positive anemia (lowest Hct 30%), positive ANA (highest titer greater than 1:15, 360; rim pattern) and elevated anti-dsDNA binding (highest 96%) with variable degrees of hypocomplementemia. One animal had granular deposition of immunoglobulin and complement at the dermal-epidermal junction of clinically normal skin the presence of immune complex-induced glomerulonephritis.

Animals↗
PubMed1996

Autoimmune cholangitis syndrome with a bias towards primary biliary cirrhosis.

The apparent coexistence of primary biliary cirrhosis (PBC) and autoimmune hepatitis in the same patient raises unresolved problems for nosology and therapy. These are exemplified by a 45-year-old Japanese woman with overlapping clinical, serological and histological features of autoimmune cholangitis and autoimmune hepatitis. The classical serological test for PBC, antimitochondrial antibody (AMA) by immunofluorescence, was atypical. By immunoblotting there was reactivity with one of the enzymes of the 2-oxo-acid dehydrogenase complex (2-OADC) family, now recognized as autoantigens responsible for AMA reactivity. Also there was reactivity by immunofluorescence for antinuclear antibodies (ANA), one showing the typical speckled pattern of anti-Sp-100 and the other the peripheral pattern of antinuclear membrane antibody, both with titres > 10(6). There was also a positive result to the lupus erythematosus (LE) cell test. Treatment with ursodeoxycholic acid was beneficial. Thus while the clinical presentation suggested the overlapping syndrome of autoimmune hepatitis and PBC, PBC eventually proved to be the likely diagnosis. We suggest that apparent cases of overlapping PBC-autoimmune cholangitis-hepatitis syndromes, after detailed testing, will mostly align with PBC.

Antibodies, Antinuclear↗
PubMed1985

ANAs in systemic rheumatic disease. Diagnostic significance.

Development of the lupus erythematosus (LE) cell test some 40 years ago opened up the world of ANAs to the clinician. Armed with the availability of a battery of tests for ANAs of different specificities, the modern clinician has the tools for increased diagnostic sensitivity in rheumatic disease and more accurate differentiation of systemic rheumatic diseases that sometimes present with confusing overlapping clinical features. The tests, however, should not be used indiscriminately, and their interpretation should always be done critically and in the context of the clinical presentation.

Antibodies, Antinuclear↗
PubMed2006

Comparative expression and distribution of c-fos, estrogen receptoralpha (eralpha), and p38alpha in the uterus of rats, monkeys, and humans.

The uterine cellular expression and distribution of c-fos, ERalpha and p38alpha was compared in humans, nonhuman primates, and rats using immunohistochemistry. ERalpha and c-fos were present in the glandular (GE) and luminal epithelial cells (LE) of humans and nonhuman primates, with differing expression patterns evident between proliferative and secretory cycle phases. In rats, the highest and lowest expression of c-fos was present during proestrus and estrus, respectively, in the LE and GE. The most intense ERalpha staining in rats was observed during proestrus in the GE, while the least intense staining was seen in the LE during proestrus. Strong LE and GE expression of p38alpha was present in rats in all stages of the estrous cycle and during the proliferative phase in both humans and nonhuman primates. No p38alpha expression was observed during the secretory phase in either humans or nonhuman primates. Our work suggests that c-fos, ERalpha and p38alpha (a) are primarily expressed during the proliferative phase, but not the secretory phase and exhibit interspecies expression variability, and (b) rats exhibit cyclic changes in the expression of c-fos and ERalpha.

Animals↗
PubMedDate not supplied

Persistent HB antigenemia: associated clinical manifestations and hepatic lesions.

The clinical, biochemical and histological features in nine asymptomatic HBs Ag carriers, 15 patients with symptomatic HBs Ag-positive chronic active hepatitis (CAH), and 29 patients with HBs antigenemia persistent for five months or longer following a documented attack of acute viral hepatitis were compared, and contrasted with those in 67 patients with HBs Ag-negative CAH. The findings were remarkably similar in all groups. However, young males predominated in all three HBs Ag-positive groups, whereas most HBs Ag-negative cases of CAH were in middle-aged women. Moreover, in the latter group, the lesions tended to be more extensive and more commonly were accompanied by smooth muscle antibody, antinuclear antibody and LE cells, and extrahepatic manifestations, such as ulcerative colitis, arthritis, pleurisy and pericarditis.

Adolescent↗
PubMedDate not supplied

Endralazine, a new peripheral vasodilator--a randomized cross-over trial against dihydralazine.

In a randomized cross-over trial in 23 patients with essential hypertension, a new peripheral vasodilator, endralazine, in a dose of 10-20 mg t.i.d. was compared with dihydralazine in a dose of 25-50 mg t.i.d. All patients also received pindolol (a beta-blocker) in a dose of 5-15 mg t.i.d. The lowest dose of both drugs was given to all patients for 2 weeks and was increased only if indicated. Endralazine was more effective than dihydralazine, but the side effects were about the same in frequency and severity, apart from flushing, which was more common with endralazine. Patients receiving endralazine in the second phase of the cross-over design continued to be treated with endralazine for a period of 10-12 months. Blood pressure control remained good during this time, and the dosage was slightly reduced. No side effects suggestive of drug-induced lupus were seen, and only borderline changes in immunological tests [antinuclear antibodies (ANA)] in one patient were seen. One patient was reported to have lupus erythematosus (LE) cells in the peripheral blood but the ANA test was negative. Endralazine appears to be a useful new drug for the treatment of hypertension.

Adolescent↗
PubMed1977

Membranous lupus nephropathy: a clinicopathologic study.

Twenty-eight patients with SLE and distinct, well-defined renal morphologic lesions of membranous nephropathy were followed up for 4 years. These patients comprised approximately 8% of the patients evaluated for SLE during a 12-year period. The patients with membranous lupus nephropathy had typical systemic features of SLE, and most of them had positive LE cell tests and ANA, low serum complement concentrations, and mildly elevated serum antinative DNA levels. Proteniuria and microscopic hematuria were usually discovered years after systemic symptoms of SLE had developed, Only two patients had slowly progressive renal failure, and most patients continued to have proteinuria. Prednisone treatment did not influence either proteinuria or renal function. In only one patient, the renal character of the disease changed drastically, demonstrating membranoproliferative glomerulonephritis. Six patients died (21%); most of these died of cardiovascular illnesses. The relatively benign and stable renal course of membranous lupus nephropathy in patients with otherwise typical SLE suggests that the renal pathogenesis is different from that of proliferative lupus nephritis.

Adolescent↗
PubMedDate not supplied

The evaluation of patients with Eales' disease.

Thirty-two patients with documented Eales' disease who have been followed-up at Johns Hopkins Hospital for periods of as long as 37 years were investigated. The previously reported association of Eales' disease with tuberculoprotein hypersensitivity was confirmed in 10 of 21 patients tested. For the first time, associated vestibuloauditory dysfunction is described. The previously reported association with multiple sclerosis was not confirmed. Eales' disease is a specific vasculopathy that can affect both the retinal and vestibuloauditory circulations. Patients with suspected Eales' disease should also be examined to rule out other diseases such as diabetes, sarcoidosis, and connective tissue diseases including systemic lupus erythematosus. This can be done by obtaining such tests as fasting blood glucose, chest radiograph, erythrocyte sedimentation rate, antinuclear antibody, rheumatoid factor, and LE cell preparation. Tuberculoprotein sensitivity status should be ascertained and active tuberculosis should be ruled out. The patient should be questioned regarding hearing or balance problems, and if a history suggestive of abnormalities is elicited, referral for vestibuloauditory function testing should be made.

Adult↗
PubMed1979

Acute sarcoidosis occurring during the course of systemic lupus erythematosus.

A diagnosis of systemic lupus erythematosus (SLE) was established in a 54-year-old woman with a compatible history, as well as patchy alopecia, anemia, arthralgias, and a positive LE cell preparation. Sixteen months later bilateral hilar and mediastinal lymph nodes appeared on chest roentgenogram. Sarcoidosis was diagnosed when hypercalcemia and noninfectious, noncaseating epithelioid granulomas were found in the skin and liver. The sarcoidosis remitted with corticosteroid therapy, but slowly advancing renal failure ultimately resulted in the patient's death. We believe the concurrence of SLE and sarcoidosis had not been previously reported in the English literature. Immune mechanisms are discussed.

Female↗
PubMed1987

Cardiac tamponade in systemic lupus erythematosus: an unusual initial manifestation.

I have described a patient in whom cardiac tamponade occurred as the initial clinical manifestation of SLE. Although pericarditis is a common clinical entity in SLE, cardiac tamponade with this disease is rare. If suspected, the diagnosis can be made by the proper selection of tests of serum and pericardial fluid, which should include the search for pericardial LE cells. This report emphasizes the importance of screening for connective tissue disease in patients with pericarditis.

Adult↗
PubMed1980

Psoriatic arthritis: a clinico-radiological study.

A less documented entity of psoriatic arthritis was studied in 238 cases of psoriasis and it was detected in 5.1 % of cases. The prevalence of arthritis was found in the population over 40 years of age and was 2.7% of the males and 8.2% of the females. Psoriatic arthritis was much more common in females (male to female ratio = 1:2) in contrast to male predominance in psoriasis (male to female ratio = 1.6:1. Skin lesions usually precede the arthritis (50%); in others the onset is synchronous (41.7%) and in few of them the arthritis may come first (8.3%). Athritis is commonly encountered with moderate to severe involvement of skin (75%). In deforming type of arthritis the psoriasis was extensive and exfoliative (17%). The nails are more frequently involved at the onset of the arthritis. Rose-Waaler Test was negative in all of them. Anaemia (41.6%) and raised ESR (25%) was a feature in the active phase of the disease. Hyperurecaemia was noted in 41.6% of cases, indicating towards the extensive involvement of skin. The importance of radiological evaluation of spine, sterno-clavicular and sacro-iliac joints in all patients is stressed. Certain unusual features noted in the presented series of cases are; calcaneal spur (17%), spondylolisthesis (8.5%) and presence of LE cells (8.3%).

Adolescent↗
PubMed1975

The significance of a positive cutaneous immunofluorescence test in systemic lupus erythematosus.

Direct cutaneous immunofluorescence microscopical examination of uninvolved skin is an important diagnostic test in systemic lupus erythematosus. Its prognostic significance is undetermined. In twenty-four patients there was an increased incidence of leukopenia, hypocomplementaemia, and LE cells in patients with positive skin immunofluorescence. Positive cutaneous immunofluorescence of uninvolved skin was correlated with the most severe forms of lupus renal disease, membranous glomerulonephritis, and diffuse proliferative glomerulonephritis.

Adolescent↗
PubMed1983

A study of familial lupus erythematosus-like disease and hereditary angio-oedema treated with danazol.

Immunological and genetic studies were performed in a family in which the mother and five siblings had hereditary angio-oedema (HAE). LE cells, antinuclear factors, antibodies to double-stranded DNA and positive direct Coombs' test were not found in any of the subjects. One female sibling had a lupus erythematosus-like illness with skin lesions of the face which responded to danazol treatment. Laboratory evidence of HAE was obtained in the entire kindred and all the siblings shared HLA haplotype AIB7 inherited from the deceased mother.

Adolescent↗