Cases reported "Sarcoidosis"

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1/33. cryptococcosis during systemic glucocorticosteroid treatment.

    cryptococcosis is an opportunistic infection caused by a fungus, cryptococcus neoformans. It is usually seen in immunocompromised patients with AIDS, leukaemia, lymphoma, sarcoidosis or immunosuppressive treatments. We describe a patient who was treated with systemic glucocorticosteroids for 4 years because of lung sarcoidosis. During the last year of treatment, a papular eruption developed which later became ulcerative. In a histopathological examination of a skin biopsy, there was granulomatous inflammation, and the disease was treated as sarcoidosis without success. After 1 year's unsuccessful treatment, another skin biopsy and skin fungal culture revealed C. neoformans. Cryptococcal antigen was found in blood and cerebrospinal fluid, too. The patient was successfully treated first with an amphotericin-B-flucytosine combination and later with fluconazole.
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2/33. Anti-dsDNA antibodies in sarcoidosis.

    BACKGROUND: sarcoidosis is a chronic multisystem disorder characterized by an exaggerated cellular immune response to antigens with the production of various antibodies including rheumatoid factor and antinuclear antibodies (ANA). The prevalence and significance of antibodies to double-stranded dna (anti-dsDNA) in sarcoid patients is unknown. The occurrence of anti-dsDNA antibodies is known to be a specific marker of systemic lupus erythematosus (SLE). sarcoidosis can occur with SLE. It is unclear if anti-dsDNA antibodies in patients with sarcoidosis signify the eventual development of SLE. OBJECTIVES: To determine the prevalence of anti-dsDNA antibodies in patients with sarcoidosis in a university hospital and their significance in predicting the diagnosis of associated SLE. methods: In a retrospective study, 34 patient files with diagnosed sarcoidosis in a university hospital during a period of 15 years were reviewed for serological markers, including ANA, anti-dsDNA, and immunoglobulin and C3 levels. The occurrence of SLE in these patients also was evaluated. RESULTS: ANA were positive in 10 of 34 of the patients screened. Two patients with sarcoidosis had antibodies to dsDNA. C3 levels in these 34 patients were an average of 87.7 /- 25.3 mg/100 mL, which is within the normal range. IgG immunoglobulin levels were an average of 2,206 /- 999 mg/100 mL, which was above normal limits. The 2 patients who were positive for anti-dsDNA had normal C3 levels and SLE did not develop during a follow-up period of 10 to 15 years. CONCLUSIONS: Anti-dsDNA antibodies may occur in patients with sarcoidosis, but their presence does not predict the subsequent development of SLE.
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3/33. Mycobacterial dna in recurrent sarcoidosis in the transplanted lung--a PCR-based study on four cases.

    sarcoidosis is a systemic granulomatous inflammation, which may be caused by mycobacteria other than M. tuberculosis complex (MOTT) in one-third of cases. A few cases of recurrent sarcoidosis in the transplanted lung have been reported. However, mycobacteria have been excluded by acid-fast stains only. We investigated four cases of recurrent sarcoidosis in lung transplant patients. Using PCR for the insertion sequence 6110 of mycobacterium tuberculosis complex and a second PCR for the mycobacterial chaperonin (65-kDa antigen coding sequence), we looked for mycobacterial dna. In three cases sequence analysis was also performed. One patient was negative for mycobacterial dna in explanted, but positive for M. tuberculosis dna in transplanted lung, qualifying this case as M. tuberculosis infection in the transplant. Three patients were negative for M. tuberculosis dna, but were positive for MOTT-dna in both explanted and transplanted lungs. In these three patients sequence identity of the amplified sequences before and after transplantation was proven, which rules out mycobacteriosis. Recurrent sarcoidosis does occur, but can only be proven by the exclusion of mycobacterial dna. In cases of recurrent MOTT-dna-positive sarcoidosis the diagnosis cannot be confirmed except by proof of sequence identity. Probably MOTT-dna-positive sarcoidosis is more likely to recur in a transplanted lung.
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4/33. Non-caseating granulomata associated with hypocellular myelodysplastic syndrome.

    Non-caseating granuloma (NCG) remains a histopathological hallmark for sarcoidosis. Although the exact mechanism for NCG formation is unknown, the pathogenesis may involve a disordered antigen presentation in the monocyte/macrophage system, functional abnormalities in activated t-lymphocytes and uncontrolled cytokine production. Similar immunological dysfunction has been described in myelodysplastic syndrome (MDS). However, the association of NCG and MDS is rarely documented. We report a case of hypocellular MDS associated with generalized NCG. Despite treatment for both sarcoidosis and tuberculosis, the patient failed to respond. A clonal myeloid disorder which was initially suppressed by T-cell immunosurveillance evolved after treatment with anti-thymocyte globulin. Although the coexistence of sarcoidosis remains a possibility, the lack of supportive clinical evidence of sarcoidosis, the abnormal appearances of the bone marrow, together with the failure to improve on high-dose steroid favour the clonal myeloid disorder as the sole pathology.
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5/33. Surgical excision of iris nodules in the management of sarcoid uveitis.

    PURPOSE: To illustrate the role of surgical removal of iris nodules (granulomas) in the management of sarcoid uveitis. STUDY DESIGN: Two interventional case reports. methods: The authors describe the clinical course of two 10-year-old males with long-standing granulomatous uveitis refractory to medical antiinflammatory and immunomodulatory therapy. Both patients were seen with iris masses, which were excised and biopsied, with findings of sarcoidosis. MAIN OUTCOME MEASURES: Control of ocular inflammation as evidenced by decrease in inflammatory cells in both anterior and posterior chambers. RESULTS: On excision of the iris masses, the ocular inflammation was controlled in both patients. CONCLUSIONS: The authors hypothesize that iris granulomas may not only be products of persistent antigenic stimulation characteristic of sarcoidosis but subsequently become foci of continued cytokine production and ocular inflammation. Total surgical removal of the iris masses may help in the diagnosis and control of sarcoid uveitis refractory to medical management.
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6/33. PET and PLAP in suspected testicular cancer relapse: beware sarcoidosis.

    A 31-year-old man previously treated with chemotherapy for metastatic testicular cancer presented with new mediastinal lymphadenopathy and peripheral lung opacities. serum tumour markers were not elevated and a PET (positron emission tomography) scan revealed increased FDG (fluoro-deoxyglucose) uptake in the lungs and mediastinum consistent with testis cancer relapse. A biopsy of a mediastinal lymph node was performed and the pathology was that of sarcoidosis. immunohistochemistry however was positive for PLAP (placental alkaline phosphatase) and negative for EMA (epithelial membrane antigen). This immunohistochemical profile raised concerns that the observed pathology represented a sarcoid reaction to micro-metastatic testicular cancer relapse. We performed immunohistochemical pathology analysis on four known cases of sarcoidosis and found the same immunohistochemical-staining pattern. This case highlights the problem of specificity when interpreting the significance of PET scans and immunohistochemical analysis in this situation. sarcoidosis, a condition that has been associated with testicular cancer, should always be considered in the differential diagnosis.
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7/33. Specific bronchoalveolar lavage fluid T cells associate with disease in a pair of monozygotic twins discordant for sarcoidosis.

    A 49-year-old Caucasian woman had an acute onset of sarcoidosis. bronchoscopy with bronchoalveolar lavage (BAL) showed a pronounced accumulation of BAL fluid CD4 T cells expressing the T-cell receptor (TCR) AV2S 3 gene. In line with this observation, the patient was HLA-DR 17 positive, previously shown to strongly correlate with lung compartmentalized AV2S3 T cells. At follow-up after recovery, reduced numbers of BAL fluid AV2S3 T cells were found. Interestingly, BAL fluid of a healthy monozygotic twin sister contained normal numbers of AV2S3 lung T cells. This report shows the T-cell repertoire of BAL fluid T cells to correlate with the disease (sarcoidosis). indicating a local and specific immune response triggered by an unknown antigen in sarcoidosis.
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8/33. Cutaneous sarcoidosis successfully treated with topical tacrolimus.

    sarcoidosis is a disorder characterized by macrophage- and T-cell-mediated responses to as yet unidentified infectious antigens or autoantigens. We describe a 62-year-old woman with a 10-year history of orange-yellow plaques of sarcoidosis on her face. Her cutaneous lesions responded to topical tacrolimus ointment after unsuccessful treatment with topical and systemic corticosteroids. No adverse effects were noted with topical tacrolimus in this patient. We discuss the mode of action by which this immunosuppressive agent may act against sarcoidosis.
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9/33. Juvenile sarcoidosis after BCG vaccination.

    We report the case of a 2-year-old boy with juvenile sarcoidosis, in whom the cutaneous lesions first arose at the site of and soon after a BCG vaccination. Juvenile sarcoidosis is rare, and the pattern of clinical features is distinct from the adult form of sarcoidosis, possibly related to immunologic development. The cause of sarcoidosis is unknown, although there is much interest in the possibility of mycobacterial species operating as antigenic stimuli to initiate the disease. This case suggests that the mycobacterium bovis present in the BGC vaccination may have been etiologically important in the development of sarcoidosis.
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10/33. Acute respiratory failure in a patient with sarcoidosis and immunodeficiency--an unusual presentation and a complicated course.

    Pulmonary sarcoidosis is usually a chronic, insidious disease resulting from granuloma formation in the lung parenchyma. The epithelioid non-caseating granulomata of sarcoidosis are the result of a T-helper1-mediated immune reaction to an unknown self or foreign antigen. We describe the case of a patient with sarcoidosis and a coexistent common variable immunodeficiency who presented with rapidly progressive respiratory failure. This unusual presentation was followed by a complicated course with recurrent pneumoccocal infections, which could be explained by the coexistence of common variable immunodeficiency. physicians should be alert to the possibility of sarcoidosis even when the clinical presentation is of acute respiratory distress syndrome (ARDS) since early treatment with steroids can be lifesaving. The detection of accompanying hypogammaglobulinemia is also crucial, as treatment with intravenous immunoglobulins (IVIG) together with steroids can improve the patient's outcome.
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