Severe breathlessness, mouth ulcers and skin blistering in a female

Gudi, V.; Ormerod, Anthony; Weir, James; Kerr, Keith; Devereux, Graham

doi:10.1183/09031936.04.00023204

Cited by 3 publications

(3 citation statements)

References 13 publications

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“…Four (3.3%) patients had no radiographic evidence of malignancy, supporting the respective reporting author’s assertion that the patient had PAMS without underlying malignancy . In five (2.4%) patients, the imaging study was used to demonstrate radiographic evidence of bronchiolitis obliterans (an associated pulmonary complication of PAMS) …”

Section: Resultssupporting

confidence: 52%

“…From the literature search, we included an additional 124 articles, reporting 208 patients with PAMS published between January 1, 1996, and August 1, 2012 . A single article reporting patients from our institution was excluded from analysis to avoid duplicate reporting .…”

Section: Resultsmentioning

confidence: 99%

“…Table outlines the number of patients who underwent diagnostic imaging studies, and the purpose and clinical utilization of the diagnostic imaging studies. Of all the patients studied in the literature review, 106 (51.0%) were reported to undergo a form of diagnostic imaging . Of these patients, 88 (83.0%) had an underlying malignancy detected or confirmed by diagnostic imaging (CT, n = 82; MR, n = 12; chest radiograph, n = 10; 18F‐FDG PET/CT, n = 4).…”

Section: Resultsmentioning

confidence: 99%

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Diagnostic imaging in paraneoplastic autoimmune multiorgan syndrome: retrospective single site study and literature review of 225 patients

et al. 2014

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Diagnostic imaging is frequently utilized in PAMS with unknown malignancy. Both conventional CT and 18F-FDG PET/CT are likely to detect the typical underlying neoplasms. Relative to conventional CT, 18F-FDG PET/CT may provide additional useful information regarding prognosis for the likely underlying malignancies, although there is a paucity of reports describing the use of this modality for this purpose.

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Section: Resultssupporting

confidence: 52%

Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

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Diagnostic imaging in paraneoplastic autoimmune multiorgan syndrome: retrospective single site study and literature review of 225 patients

et al. 2014

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Achievement of the longest survival of paraneoplastic pemphigus with bronchiolitis obliterans associated with follicular lymphoma using R-CHOP chemotherapy

et al. 2017

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Paraneoplastic pemphigus (PNP) is a rare, fatal, paraneoplastic autoimmune mucocutaneous blistering disease, commonly associated with lymphoproliferative disorders, including malignant lymphomas. Lymphoproliferative disorders associated with PNP are sometimes associated with a serious lung complication, bronchiolitis obliterans (BO). Due to its rarity, guidelines for the management of PNP have not been established. Furthermore, most patients die within 1 year. Here we report the successful treatment of lymphoma-associated PNP and BO using R-CHOP chemotherapy. A 53-year-old Japanese man was admitted to our hospital for severe erosive stomatitis. Computed tomography and positron emission tomography showed multiple lymphadenopathies. He was diagnosed with follicular lymphoma (Ann Arbor stage IVA) and PNP-related BO. The patient underwent six cycles of R-CHOP and an additional cycle of rituximab. Both the erosive stomatitis and the obstructive lung disease persisted, but complete response of the follicular lymphoma was achieved. The patient survived 27 months after diagnosis. Although he died from progressive respiratory failure due to BO, we note that this patient achieved the longest survival of any reported case of PNP-related BO associated with a lymphoproliferative disorder. The present case suggests that intensive immunochemotherapy for underlying lymphoma may improve the prognosis in patients with PNP-related BO associated with lymphoma.

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Challenges in pulmonary fibrosis {middle dot} 2 : Bronchiolocentric fibrosis

Cordier

2007

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Severe breathlessness, mouth ulcers and skin blistering in a female

Cited by 3 publications

References 13 publications

Diagnostic imaging in paraneoplastic autoimmune multiorgan syndrome: retrospective single site study and literature review of 225 patients

Diagnostic imaging in paraneoplastic autoimmune multiorgan syndrome: retrospective single site study and literature review of 225 patients

Achievement of the longest survival of paraneoplastic pemphigus with bronchiolitis obliterans associated with follicular lymphoma using R-CHOP chemotherapy

Challenges in pulmonary fibrosis {middle dot} 2 : Bronchiolocentric fibrosis

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