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At least 343 records · Page 19Linked to original sources
PubMed2001

Thalidomide and its dermatologic uses.

This article reviews how the drug thalidomide can be used for various dermatologic disorders. Also included is a short discussion of the chemistry and physiologic mechanisms of thalidomide. Thalidomide, now available again in the United States, is another drug that can be considered by dermatologists for some severe, unusual disorders.

Acquired Immunodeficiency Syndrome↗
PubMed1991

Systemic lupus erythematosus, dermatomyositis, scleroderma, vasculopathies, and other connective tissue disorders in children.

The diverse disorders discussed in this review share the underlying features of multisystem involvement and vasculitis as a part of their pathophysiology. A review of morbidity seen in childhood-onset systemic lupus erythematosus reveals the need for careful monitoring for complications secondary to treatment and infections. Current understanding of the pathophysiology and risks of developing the neonatal lupus syndrome are reviewed, with the important recognition of the association of specific maternal autoantibodies with affected offspring. A review of current management issues in the treatment of juvenile dermatomyositis is discussed. Kawasaki disease, one of the most common childhood vasculitides, continues to provoke interest in the areas of accurate diagnosis, potential etiologic role of common viruses, management, and late morbidity. Interesting case reports of children with unusual features of the more rarely described disorders of Sjögren's syndrome, scleroderma, and Behçet's syndrome are discussed.

Antiphospholipid Syndrome↗
PubMed2005

Adamantiades-Behçet's disease, deep venous thrombosis and anticardiolipin antibodies: report of two cases.

Vascular thrombosis was found in different proportions of patients with Adamantiades-Behçet's disease (ABD), depending on the ethnicity of the population under study. Various thrombophilic factors, including the levels of anticardiolipin antibodies (ACA), were investigated for their role in the thrombotic process with conflicting results. The prevalence of ACA varies considerably in different studies, but their presence has not been associated with increased risk for vascular thrombosis. We present two cases with ABD, deep venous thrombosis (DVT) and elevated levels of ACA that fulfil the criteria for both ABD and antiphospholipid syndrome (APS).

Adult↗
PubMed1995

Cerebral venous sinus thrombosis and antiphospholipid antibodies.

We report two cases of cerebral venous sinus thrombosis associated with an antibody to phospholipids, namely the lupus anticoagulant. Both patients later developed further immunologically mediated conditions. The importance of screening for the lupus anticoagulant in addition to anticardiolipin antibodies in this condition and the need for follow-up of such patients is discussed.

Adult↗
PubMed2001

Total occlusion of inferior vena cava in a patient with antiphospholipid antibody syndrome associated with behçet's disease.

Behçet's disease frequently involves the venous system, usually affecting small vessels, but sometimes large vessels such as the vena cava. Antiphospholipid antibody syndrome is associated with an increased incidence of arterial and venous thrombosis. A 29-year-old male with Behçet's disease developed bilateral leg edema secondary to thrombotic occlusion of the inferior vena cava. Laboratory tests revealed positive antiphospholipid antibodies and lupus anticoagulant. Treatment with steroid and warfarin subsequent to intravenous administration of uro-kinase resulted in improvement of symptoms. The association of antiphospholipid antibody syndrome and Behçet's disease may have caused the total thrombotic occlusion of the vena cava in this case.

Adult↗
PubMed2000

[Inflammatory bowel disease in children].

Beside Crohn's disease (CD) and ulcerative colitis (UC), paediatric chronic inflammatory bowel diseases encompass variety of different inflammatory conditions: allergic colitis in infancy, colitis of hypereosinophilic syndrome, Behcet colitis, microscopic colitides etc. Paediatric CD characterises predominance of inflammatory and obstructive disease variant. Because of their growth suppression an early introduction of azathioprine in steroid dependent and steroid refractory cases is frequently indicated. In teenage period resection procedures may be an elective and initial therapeutic option due to necessity of proper pubertal mental and physical development. Paediatric UC frequently manifests initially as pancolitis what indicates higher rate of colectomy rate. Therapy is still dispute about an optimal surgical procedure: restorative proctocolectomy or ileo-anal pull through techniques. Allergic colitis in infancy may mimic ulcerative colitis and rises a possibility of diagnostic and therapeutic confusions. Its treatment is strictly nutritive.

Child↗
PubMed2005

B-cell depletion for rheumatic diseases: where are we?

Immune system dysfunction is common to rheumatic disorders, with rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE) being classic examples. Altered development and function of B cells may play a prominent role. B-cell abnormalities also occur in other rheumatic diseases, eg, Sjogren's syndrome, Behcet's disease, antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, and dermatomyositis. Hence, B-cell depletion has been investigated as a therapeutic option. Clinical trials in RA and SLE have shown that rituximab, an anti-CD20 monoclonal antibody, can profoundly reduce disease activity and is generally well tolerated. Reports of rituximab treatment for ANCA-associated vasculitis and dermatomyositis are also promising. These encouraging results validate the strategy of B-cell depletion in various rheumatic diseases. B-cell depletion with rituximab is under study in larger clinical trials for the purposes of regulatory approval to define more closely its place in RA and SLE treatment paradigms, and smaller clinical trials are ongoing or planned in associated inflammatory diseases.

B-Lymphocytes↗
PubMed1994

[Intractable vasculitis syndromes--incidence and epidemiology].

Incidence and epidemiology of various diseases related to vasculitis were mainly reviewed from recent studies carried out in the Research Committee of Intractable Vasculitis Syndrome supported by the Ministry of Health and Welfare of Japan. A relative higher proportion of patients with aortitis syndrome (Takayasu's arteritis), Buerger's disease was reported in Asia compared to Europe and the United States. Today, the incidence of these diseases is considered to indicate a trend to decrease in Japan. However, the number of patients studied by the Committee remained almost unchanged. The patients are reported from all districts of Japan, though the incidences varied with the prefecture. The Research Committee is continuing further careful epidemiological investigations by established diagnostic criteria from year to year.

Adolescent↗
PubMed1989

Pulmonary involvement in Behçet's syndrome.

We present a case of Behcet's syndrome with unusual pulmonary manifestations. A woman presented five years previously with oral, genital and ocular lesions found on biopsy to be consistent with Behcet's syndrome. While on prednisolone, she complained of nonpleuritic chest pain. Clinical features of active Behcet's syndrome were absent. A previously normal chest x-ray film showed multiple intrapulmonary lesions. Laboratory abnormalities were consistent with findings seen in phases of disease activity. Bronchoscopy showed ulceration of the bronchial mucosa, but no evidence of opportunistic infection or neoplasia. Pulmonary angiography excluded vaso-occlusive abnormalities. A provisional diagnosis of pulmonary involvement with Behcet's syndrome was made and the patient responded to further immunosuppressive therapy.

Behcet Syndrome↗
PubMed1991

Association between the 65-kilodalton heat shock protein, Streptococcus sanguis, and the corresponding antibodies in Behçet's syndrome.

The etiology of Behcet's syndrome (BS) is unknown, but a number of streptococcal species have been implicated. A hypothesis was postulated that a shared antigen, such as a stress protein, might account for some of these findings. Indeed, a rabbit antiserum against a 65-kDa heat shock protein of Mycobacterium tuberculosis revealed a corresponding 65-kDa band with all six Streptococcus sanguis strains examined and S. pyogenes but not with S. salivarius. By applying a panel of nine monoclonal antibodies to the mycobacterial 65-kDa heat shock protein, an approximately 65-kDa antigen was identified in the uncommon serotypes of S. sanguis ST3 and H.83 and one with a different Mr was identified in KTH-1 and S. pyogenes. Monoclonal antibodies Y1.2, C1.1, II H9, and ML30, which reacted with these streptococci, recognize residues 11 to 27, 88 to 123, 107 to 122, and 276 to 297 of the 65-kDa heat shock protein, respectively, suggesting that these residues are conserved among some uncommon serotypes of S. sanguis and S. pyogenes. Immunoblot analyses of sera from patients with BS for immunoglobulin A (IgA) and IgG antibodies revealed bands of 65 to 70 kDa with the mycobacterial heat shock protein, S. sanguis strains, and S. pyogenes, although these reactivities were also found to a lesser extent in controls. A 65- to 70-kDa band was found more frequently with S. sanguis KTH-2 or KTH-3 and IgA in serum from patients with BS than with serum from controls (P less than 0.02). Antibodies in serum were then studied by a radioimmunoassay, and in patients with BS this revealed significantly raised IgA antibodies to the recombinant 65-kDa mycobacterial heat shock protein and to soluble protein extracts of S. sanguis ST3, KTH-1, KTH-2, and KTH-3. Whereas significant anti-65-kDa heat shock protein and anti-S. sanguis ST3 antibodies were also found in sera from patients with rheumatoid arthritis and recurrent oral ulcers, the anti-S. sanguis KTH-1, KTH-2, and KTH-3 antibodies were confined to BS. The results are consistent with the hypothesis that some of the streptococcal antigens are associated with heat shock or stress proteins, which will need to be formally established by isolating heat shock proteins from streptococci.

Amino Acid Sequence↗
PubMed1985

Chronic active hepatitis and Behçet's syndrome.

In a patient with Behcet's syndrome the finding of five times the normal level of serum transaminase accompanied by weakness may be correlated to the syndrome itself. We performed needle biopsy of the liver in our patient which showed piecemeal necrosis and portal lympho-monocytic infiltration extending into the lobule. It is interesting that HLA B5 and B27 antigens were present. Simultaneous occurrence of chronic active hepatitis and Behcet's syndrome may imply the same autoimmune pathogenesis, as we suggest in this work.

Alanine Transaminase↗