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

Lehman, Vance T.; Barrick, Benjamin J.; Pittelkow, Mark R.; Peller, Patrick J.; Camilleri, Michael; Lehman, Julia S.

doi:10.1111/ijd.12603

Cited by 19 publications

(14 citation statements)

References 152 publications

(270 reference statements)

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“…The clinical features of PNP include stomatitis, mucositis, and skin lesions. Furthermore, PNP is frequently associated with hematologic neoplasms, including non-Hodgkin lymphoma, chronic lymphocytic leukemia, and CD [21,22]. In this series of CD cases, 9 were considered to have PNP.…”

Section: Discussionmentioning

confidence: 98%

Clinical characteristics and prognosis of castleman disease patients in a Chinese hospital: paraneoplastic pemphigus is an independent risk factor

Hua

Liang

Yang

et al. 2020

Preprint

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Background Castleman disease (CD) is a rare lymphoproliferative disorder that has had limited clinical research. This study aims to detect the clinical manifestations, pathological features, and prognostic factors of this disease.Methods This study retrospectively analyzed the information of 54 patients with CD hospitalized in a single centre. A Cox regression model was employed to perform univariate analysis and multivariate analysis in order to identify independent prognostic factors for survival.Results Based on clinical classification, 26 patients (55.6%) had unicentric CD (UCD) and 28 patients (44.4%) had multicentric CD (MCD). Moreover, pathological classification identified 32 cases (59.3%) with hyaline vascular variant (HV), 3 (5.6%) with mixed cellular variant (Mix), and 19 (35.2%) with plasmacytic variant (PC). The MCD patients commonly exhibited clinical signs and symptoms, including fever, splenomegaly, and pleural effusion and/or ascites. Several clinical complications, such as liver injury, anemia, and polyradiculoneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, skin changes syndrome (POEMS) were more common in MCD patients. Univariate analysis showed that presence of paraneoplastic pemphigus (PNP) and elevated C-reactive protein (CRP) were unfavorable factors relating to CD patient survival. Multivariate analysis identified the presence of PNP as an independent prognostic factor in patients with CD.Conclusions This study provided a panoramic elaboration of CD cases and showed the presence of PNP was an independent unfavorable factor.

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Section: Discussionmentioning

confidence: 98%

Clinical characteristics and prognosis of castleman disease patients in a Chinese hospital: paraneoplastic pemphigus is an independent risk factor

Hua

Liang

Yang

et al. 2020

Preprint

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“…PNP is associated with underlying neoplasms, the most frequent of which are hematologic malignancies. Previous studies revealed that non-Hodgkin lymphoma (about 40%) is the most frequent neoplasm, followed by Castleman disease (15~37%) and chronic lymphocytic leukemia (CLL) (7~18%) (10, 11, 20). Castleman disease has been reported as the most frequent neoplasm in Korea and China (21, 22), suggesting that the incidence of associated neoplasms vary by ethnicity.…”

Section: Disease Manifestationsmentioning

confidence: 99%

“…Given the fact that Castleman disease has an extremely low incidence in the general population, cases of PNP with Castleman disease are highly frequent. A minor fraction of neoplasms associated with PNP represents non-hematologic neoplasms, including neoplasms originating from the thymus (e.g., thymoma), sarcoma, malignant melanoma, and various epithelial-origin carcinomas (e.g., adenocarcinoma and squamous cell carcinoma) (10, 14, 20, 24, 25). Some cases of PNP were diagnosed before an underlying malignancy was detected (26–28).…”

Section: Disease Manifestationsmentioning

confidence: 99%

Paraneoplastic Pemphigus: Paraneoplastic Autoimmune Disease of the Skin and Mucosa

Kim

2019

Front. Immunol.

172

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Paraneoplastic pemphigus (PNP) is a rare but life-threatening mucocutaneous disease mediated by paraneoplastic autoimmunity. Various neoplasms are associated with PNP. Intractable stomatitis and polymorphous cutaneous eruptions, including blisters and lichenoid dermatitis, are characteristic clinical features caused by humoral and cell-mediated autoimmune reactions. Autoreactive T cells and IgG autoantibodies against heterogeneous antigens, including plakin family proteins and desmosomal cadherins, contribute to the pathogenesis of PNP. Several mechanisms of autoimmunity may be at play in this disease on the type of neoplasm present. Diagnosis can be made based on clinical and histopathological features, the presence of anti-plakin autoantibodies, and underlying neoplasms. Immunosuppressive agents and biologics including rituximab have been used for the treatment of PNP; however, the prognosis is poor due to underlying malignancies, severe infections during immunosuppressive treatment, and bronchiolitis obliterans mediated by autoimmunity. In this review, we overview the characteristics of PNP and focus on the immunopathology and the potential pathomechanisms of this disease.

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“…In those without a prior diagnosis, workup often includes imagining. A recent analysis conducted at Mayo Clinic with 17 patients from their institution and 208 from a literature review suggested that 18 F-FDG PET/CT imaging was equal to CT in detecting malignancy; however, 18 F-FDF PET/CT was felt to better facilitate staging of lymphoma and guidance of subsequent biopsy procedures [60]. Prognosis is generally considered to be poor with mortality rates of 75-90 % quoted historically [61].…”

Section: Paraneoplastic Pemphigusmentioning

confidence: 99%

Skin Manifestations of Internal Disease in Older Adults

Markus¹,

Perry²,

Lear³

2015

Curr Geri Rep

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The skin is the largest and most visible organ in the body and can give diagnostic clues of systemic illness. These clues often become obscured through the normal aging process. The purpose of this review is to give an overview of the normal skin changes seen with aging and then discuss new findings with regards to cutaneous signs of systemic disease in the elderly. We performed a literature search on cutaneous manifestations of internal disease and limited our search to articles published within the last 5 years. We then narrowed down the articles on conditions that primarily affect the adults over the age of 65; conditions commonly seen in the elderly as well as new findings which we felt were of great significance. Ultimately, we hope to update the reader on new cutaneous diagnostic tools of internal disease.

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

Cited by 19 publications

References 152 publications

Clinical characteristics and prognosis of castleman disease patients in a Chinese hospital: paraneoplastic pemphigus is an independent risk factor

Clinical characteristics and prognosis of castleman disease patients in a Chinese hospital: paraneoplastic pemphigus is an independent risk factor

Paraneoplastic Pemphigus: Paraneoplastic Autoimmune Disease of the Skin and Mucosa

Skin Manifestations of Internal Disease in Older Adults

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