Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Five to 40% of patients with agammaglobulineamia develop joint manifestation consisting of septic arthritis or aseptic arthritis of unclear pathogenesis. Intravenous gamma-globulin therapy seems effective on the better condition but is burden-some and expensive. We report the case of a 31-year old woman, having a common variable immunodeficiency that has a chronic oligoarthritis seronegative and not warping. Substitutive treatment with gamma-globulins resulted in a poor improvement. Blood Lymphocyte immunotyping ans analysis of synovial fluid showed high levels of CD8 with a low CD4/CD8 ratio. Synovial biopsy revealed a non specific synovitis with an lymphoid aggregates with a significant predominance of CD8 cells and suspicion of mycoplasm, only after centrifugation, justifying PCR techniques. Doxycycline therapy was effective in relieving the joint symptoms with six month recession.
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PMID:[Efficacy of cyclines in agammaglobulinemia arthritis]. 1266 20

Reiter's syndrome is one of the reactive forms of seronegative spondyloarthropathies. Various therapies used in the management of Reiter's syndrome are nonsteroidal antiinflammatory drugs (NSAIDs), antibiotics, and disease-modifying antirheumatic drugs (DMARDs) such as sulfasalazine (SSZ) or methotrexate (MTX). There is only one case report of successful treatment of Reiter's syndrome with tumor necrosis factor-alpha (TNF-alpha) blockers in human immunodeficiency virus (HIV) patient (Gaylis N, 2003, J Rheumatol 30(2):407-411 Feb). We hereby report a case of Reiter's syndrome treated successfully with infliximab, an anti-TNF-alpha chimeric monoclonal antibody. A 28-year-old white male presented with painful swelling of right elbow and ankle joints, urethritis. and lesions involving skin of soles of feet and penis. Detailed work-up of sexually transmitted diseases (STDs), HIV, and systemic etiology were negative. Despite aggressive treatment with antibiotics, NSAIDS, prednisone, and MTX for 3 months, he had persistent synovitis and worsening of skin lesions. He was then treated with infliximab 200 mg intravenously at weeks 0, 2, 6, and 14 weeks which resulted in complete resolution of arthritis and skin lesions within 6 weeks of infliximab therapy.
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PMID:Successful use of infliximab in the treatment of Reiter's syndrome: a case report and discussion. 1764 85

Infectious endocarditis (IE) is an inflammatory disease of cardiac valves and endocardium of different origin. Subacute IE is a specific form of sepsis associated with the presence of the site of infection in the heart responsible for recurrent septicemia, embolism, and progressive changes in the immune system leading to nephritis, vasculitis, synovitis, and polyserositis. This form develops in response to a low-virulent pathogen (e.g.fungi) or as a result of inefficient antibacterial therapy. A patient is described presenting with fibrous body, aortic and mitral valve infection by the alga Prototheca wickerhamii associated with primary (myeloperoxidase) immunodeficiency. Recent data on diagnostics and treatment of subacute IE are presented.
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PMID:[Subacute infectious endocarditis of unusual etiology: peculiarities of diagnostics and treatment]. 2193 71

Dear Editor, Syphilis is an infection caused by Treponema pallidum. Without treatment, it goes through the following stages: primary, secondary, latent, and tertiary (1). The clinical picture of secondary syphilis is very variable (2,3). We present two rare cases of secondary syphilis, one with nodular lesions initially considered to be lymphoma and second with periostitis, which was initially interpreted as an osteoma. To date, only 15 cases with nodular lesions and 10 cases with periostitis in secondary syphilis have been reported in the literature. The first patient was a 59 year old man who presented in a private practice with nodular lesions on the face and axillary and inguinal folds (Figure 1, a, b). The initial diagnostic consideration was lymphoma. A biopsy specimen was taken, and the histopathological features revealed epidermal hyperplasia with papillomatosis, minimal spongiosis with many neutrophils and with a marked inflammatory infiltrate in dermis, consisting of lymphocytes, plasma cells, and neutrophils; the diagnosis of interfaced dermatitis was established (Figure 1, d, e). After one month, the patient presented to our clinic with numerous nodular lesions, some of them painful, located on the trunk and intertriginous folds, including the intergluteal cleft - the lesions in this area being suggestive of condylomata lata (Figure 1, c). The diagnosis of secondary syphilis was taken into consideration, and screening serum tests were performed and found reactive: a Venereal Diseases Research Laboratory (VDRL) titer of 1:64 and Treponema pallidum Hemaglutination Assay (TPHA) titer of 1:80. Hepatitis and anti-human immunodeficiency virus (HIV) antibodies serology was negative. The biopsy was repeated and showed the same histopathological changes. In addition, Warthin-Starry staining was performed, revealing the presence of some spiral micro-organisms in the dermis corresponding to Treponema pallidum (Figure 1, f). A diagnosis of secondary syphilis was established, and the patient was treated with benzathine penicillin G 2.4 million units by intramuscular injection once a week for 2 consecutive weeks. The skin lesions regressed within 1 month, and serological tests showed a VDRL titer of 1:8 3 months after treatment. The second patient was a homosexual male, 35 years old, diagnosed with HIV infection, stage B2. He presented with bone pain in the calves and forearms, with insidious onset. He also presented with an associated erythematous maculo-papular rash on the trunk and limbs and generalized lymphadenopathy (Figure 2, a, b). The tibial crest and radius were sensitive to palpation. A right leg radiography was performed, raising suspicion of osteoid osteoma. The CT scan excluded the diagnosis of osteoma; taking into account the epidemiological context, the diagnosis of syphilis was suspected. The diagnosis was confirmed by leg ultrasound examination (2D US) which showed thickening of the compact tibial bone associated with subperiosteal destructive and proliferative changes (Figure 2, c, d) and by serology for syphilis: the VDRL titer was 1:32 and the TPHA titer was 1:80. The patient was treated with benzathine penicillin 2.4 million units, once a week, for 2 consecutive weeks, with clinical improvement. Syphilis continues to be a serious public health problem worldwide, even if it is a controllable disease due to diagnostic tests and effective and accessible treatment. According to the World Health Organization in 2008, the estimated number of new cases of sexually transmitted diseases in adults with syphilis is 10.6 million cases (4). The cases presented in this paper were characterized by unusual manifestations, requiring good collaboration between the dermatologist and other specialties. In the first case, the diagnosis of secondary syphilis was confirmed by positive serological, clinical, and histopathological findings. The main differential diagnosis of nodular syphilis includes lymphoma, sarcoidosis, Kaposi's sarcoma, atypical mycobacteriosis, deep fungal infections, leprosy, tuberculosis, leishmaniasis, and lymphomatoid papulosis (5). Another important differential diagnosis is between secondary and tertiary syphilis, especially when ulcerating nodules are present. Tertiary syphilis is characterized by unilateral, deep ulcerating nodules with necrotizing granulomas (6). Bone involvement during syphilis is mainly represented by polyarthritis, synovitis, osteitis, and periostitis (7,8). Syphilitic periostitis is characterized by localized or diffuse pain, particularly during the night, which is relieved by movement. The skull, the shoulder girdle, and the long bones are the most common sites of involvement (9). In conclusion, we presented two different cases of secondary syphilis that contribute to the clinical experience of rare cases presented in the literature, raising the awareness of dermatologists and other specialists about less specific clinical aspects of syphilis.
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PMID:Unusual Manifestations of Secondary Syphilis: Case Presentations. 2998 79

Imbalance of Treg/Th17 and chronic synovitis characterized by the recruitment and infiltration of inflammatory cells are the typical features of rheumatoid arthritis (RA). IL-6 promotes the differentiation and function of Th17 cells, which contributes to the imbalance of Treg/Th17 and aggravates lymphocytic infiltration in joints. DC32, a dihydroartemisinin derivative, was found to have anti-inflammatory and immunosuppressive activities in previous study. The aim of this study is to evaluate the effects and mechanisms of DC32 in immunodeficiency and inflammatory infiltration of RA. In vivo, the antirheumatic effect of DC32 was evaluated in a collagen-induced arthritis (CIA) mouse model in DBA/1 mice. The percentages of Treg and Th17 and transcription of IL-6 in the spleen were assayed. In vitro, a coculture system of ConA-activated lymphocytes and fibroblast-like synoviocytes (FLSs) from rat with adjuvant arthritis (AA) was established. The effects and mechanisms of DC32 on synovitis were investigated. It was shown that DC32 inhibited footpad swelling and lymphocytic infiltration in mice with CIA and significantly restored the Treg/Th17 balance by reducing the transcription of IL-6 in splenocytes. DC32 significantly inhibited the lymphocyte-induced invasion and migration of FLSs by decreasing the secretion of MMPs (MMP-2, MMP-3) in vitro. DC32 also reduced the transcription of chemokines (CXCL12, CX3CL1) and IL-6 in FLSs, as well as IL-6 levels in the supernatant. These results demonstrated that DC32 may attenuate RA by restoring Treg/Th17 balance and inhibiting lymphocytic infiltration through downregulation of the expression and transcription of IL-6. This study supports the potential of DC32 to down-regulate IL-6 for the treatment of RA and other related autoimmune diseases.
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PMID:Dihydroartemisinin derivative DC32 attenuates collagen-induced arthritis in mice by restoring the Treg/Th17 balance and inhibiting synovitis through down-regulation of IL-6. 3033 38


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