Comparative study of five serological assays for the diagnosis of paraneoplastic pemphigus

Kelly, Shane; Culican, Suzanne; Silvestrini, Roger; Vu, Jacinta; Schifter, Mark; Fulcher, David A.; Lin, Ming‐Wei

doi:10.1097/pat.0000000000000196

Cited by 15 publications

(6 citation statements)

References 13 publications

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“…In 2009, Probst et al created a new ELISA based on the recombinant N-terminus of envoplakin, showing a sensitivity of 82% and a specificity of ≥98% [ 69 ]. However, it has been reported that ELISA appeared to lack sensitivity in a multi-assay comparison across different assays, because antigen-specific techniques, such as envoplakin ELISA, detect only one of a range of autoantibodies potentially responsible for the disease [ 70 ].…”

Section: Immunological Studiesmentioning

confidence: 99%

Paraneoplastic Pemphigus: Insight into the Autoimmune Pathogenesis, Clinical Features and Therapy

Paolino

Didona

Magliulo

et al. 2017

IJMS

111

195

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Paraneoplastic pemphigus is a rare autoimmune skin disease that is always associated with a neoplasm. Usually, oral, skin, and mucosal lesions are the earliest manifestations shown by paraneoplastic pemphigus patients. The pathogenesis of paraneoplastic pemphigus is not yet completely understood, although some immunological aspects have been recently clarified. Because of its rarity, several diagnostic criteria have been proposed. Besides, several diagnostic procedures have been used for the diagnosis, including indirect immunofluorescence, direct immunofluorescence, and ELISA. We reviewed the most recent literature, searching on PubMed “paraneoplastic pemphigus”. We included also papers in French, German, and Spanish. We found 613 papers for “paraneoplastic pemphigus”. Among them, 169 were review papers. Because of its varying clinical features, paraneoplastic pemphigus still represents a challenge for clinicians. Furthermore, diagnosis and management of paraneoplastic pemphigus requires close collaboration between physicians, including dermatologist, oncologist, and otorhinolaryngologist.

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Section: Immunological Studiesmentioning

confidence: 99%

Paraneoplastic Pemphigus: Insight into the Autoimmune Pathogenesis, Clinical Features and Therapy

Paolino

Didona

Magliulo

et al. 2017

IJMS

111

195

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“…The spectrum of IgG autoantibodies is more diverse in PNP, including IgG autoantibodies against Dsg 1, Dsg 3, desmoplakin 1, desmoplakin 2, Dsc 1, Dsc 3, envoplakin, periplakin, plectin, BP180, BP230, and the protease inhibitor, alpha-2-macroglobulin-like-1 (6, 80). However, ELISA lacks sensitivity in PNP patients due to the wide range of autoantigens targeted by Ig autoantibodies (81).…”

Section: Introductionmentioning

confidence: 99%

Pemphigus: Current and Future Therapeutic Strategies

et al. 2019

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Pemphigus encompasses a heterogeneous group of autoimmune blistering diseases, which affect both mucous membranes and the skin. The disease usually runs a chronic-relapsing course, with a potentially devastating impact on the patients' quality of life. Pemphigus pathogenesis is related to IgG autoantibodies targeting various adhesion molecules in the epidermis, including desmoglein (Dsg) 1 and 3, major components of desmosomes. The pathogenic relevance of such autoantibodies has been largely demonstrated experimentally. IgG autoantibody binding to Dsg results in loss of epidermal keratinocyte adhesion, a phenomenon referred to as acantholysis. This in turn causes intra-epidermal blistering and the clinical appearance of flaccid blisters and erosions at involved sites. Since the advent of glucocorticoids, the overall prognosis of pemphigus has largely improved. However, mortality persists elevated, since long-term use of high dose corticosteroids and adjuvant steroid-sparing immunosuppressants portend a high risk of serious adverse events, especially infections. Recently, rituximab, a chimeric anti CD20 monoclonal antibody which induces B-cell depletion, has been shown to improve patients' survival, as early rituximab use results in higher disease remission rates, long term clinical response and faster prednisone tapering compared to conventional immunosuppressive therapies, leading to its approval as a first line therapy in pemphigus. Other anti B-cell therapies targeting B-cell receptor or downstream molecules are currently tried in clinical studies. More intriguingly, a preliminary study in a preclinical mouse model of pemphigus has shown promise regarding future therapeutic application of Chimeric Autoantibody Receptor T-cells engineered using Dsg domains to selectively target autoreactive B-cells. Conversely, previous studies from our group have demonstrated that B-cell depletion in pemphigus resulted in secondary impairment of T-cell function; this may account for the observed long-term remission following B-cell recovery in rituximab treated patients. Likewise, our data support the critical role of Dsg-specific T-cell clones in orchestrating the inflammatory response and B-cell activation in pemphigus. Monitoring autoreactive T-cells in patients may indeed provide further information on the role of these cells, and would be the starting point for designating therapies aimed at restoring the lost immune tolerance against Dsg. The present review focuses on current advances, unmet challenges and future perspectives of pemphigus management.

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“…Good clinicopathologic correlation is key to the timely diagnosis of PNP (Figure 3), supported by the detection of PNP antibodies on testing that includes IIF on rat bladder substrate in preference to other serological assays 11 . Tissue histopathology and DIF definitively establishes the diagnosis of PNP, and should prompt further investigation for an underlying neoplasm, even in the absence of suggestive symptoms.…”

Section: Discussionmentioning

confidence: 95%

Paraneoplastic pemphigus: Diagnostic mimics, confounders and management challenges in a series of five long‐term survivors from a single centre

Emerson

Schifter

Dai

et al. 2022

Skin Health and Disease

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We present a series of five cases who presented to our institution with treatment‐refractory mucosal ulceration, all of whom were subsequently diagnosed with paraneoplastic pemphigus (PNP). This case series highlights the diagnostic and treatment considerations for PNP – in particular, the steroid‐dependent, recalcitrant, polymorphic manifestations; the combination of histopathological and clinical findings that may overlap with clinically similar diseases, for example, pemphigus vulgaris and lichen planus; the importance of immunopathological findings for its diagnosis, and the need for surveillance and management of life‐threatening bronchiolitis obliterans.

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Comparative study of five serological assays for the diagnosis of paraneoplastic pemphigus

Cited by 15 publications

References 13 publications

Paraneoplastic Pemphigus: Insight into the Autoimmune Pathogenesis, Clinical Features and Therapy

Paraneoplastic Pemphigus: Insight into the Autoimmune Pathogenesis, Clinical Features and Therapy

Pemphigus: Current and Future Therapeutic Strategies

Paraneoplastic pemphigus: Diagnostic mimics, confounders and management challenges in a series of five long‐term survivors from a single centre

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