2013
DOI: 10.4021/jmc1202w
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STILL out of Breath?

Abstract: Adult onset Still's disease (AOSD) is a rare inflammatory syndrome mostly seen in young adults. Known for its wide range of clinical manifestations, AOSD often presents with non-remitting systemic signs and symptoms. Many rare case associations have been described with AOSD, but only few with pulmonary hypertension (PAH). We are presenting a sixth known case of a young female adult with AOSD and PAH in the literature.

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Cited by 2 publications
(3 citation statements)
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“…A Mycobacterium tuberculosis-specific ELISPOT (T-SPOT.TB) assay was performed, and the results were negative. The number of nucleated cells in the pleural effusion was 1,760×10 6 /L, and the percentages of mononuclear cells and multinucleate cells were 80% and 20%, respectively. Her adenosine deaminase level was within the normal range.…”
Section: Case Reportmentioning
confidence: 93%
“…A Mycobacterium tuberculosis-specific ELISPOT (T-SPOT.TB) assay was performed, and the results were negative. The number of nucleated cells in the pleural effusion was 1,760×10 6 /L, and the percentages of mononuclear cells and multinucleate cells were 80% and 20%, respectively. Her adenosine deaminase level was within the normal range.…”
Section: Case Reportmentioning
confidence: 93%
“…PAH is a very rare complication of AOSD, but its prevalence is probably underestimated because only symptomatic case reports have been documented (11). To our knowledge, 15 cases have been reported (110)(111)(112)(113)(114)(115)(116)(117)(118)(119)(120)(121)(122)(123). Age of onset ranged from 18 to 41 years old.…”
Section: Pulmonary Arterial Hypertensionmentioning
confidence: 99%
“…Anakinra was efficacious (in combination with vasodilators and corticosteroids, and either azathioprine or cyclosporine) in two case reports (one case also had RHL) (110,117). Failure was reported in one patient (121), and 3 patients (one died) showed worsening after its introduction (114,118,123). However, with the critical and unstable nature of this complication, drawing robust conclusions about the specific anakinra role in these unfavorable evolutions is difficult.…”
Section: Pulmonary Arterial Hypertensionmentioning
confidence: 99%