Paraneoplastic Pemphigus (Paraneoplastic Autoimmune Multiorgan Syndrome) Expanded Antibody Panel by IIF With ELISA

The methodology is indirect immunofluorescence (IIF) of patient serum with rodent substrates including rat bladder, mouse bladder, mouse heart, and mouse liver to detect characteristic IgG antibody reactivity: simple columnar epithelial cell surface and basement membrane zone in bladders, intercalated discs in heart, and portal tracts in liver, and enzyme-linked immunosorbent assay (ELISA) to detect IgG antibodies to envoplakin. Monkey esophagus substrate is included if other concurrent IIF testing does not.

This test should be distinguished from antibody testing of cerebral spinal fluid (CSF) for paraneoplastic neurologic syndromes; 3004510, 3004512, 3004517 are different tests.

Paraneoplastic pemphigus (PNP), also known as paraneoplastic autoimmune multiorgan syndrome (PAMS), is a rare paraneoplastic disease that affects patients of all ages, is associated with lymphoproliferative disorders/malignancies and demonstrates clinical features of severe pemphigus with a high mortality rate.

Patients with PNP/PAMS develop serum antibodies to multiple epithelia (simple, columnar, transitional) with several possible epithelial antigen targets. Envoplakin is one of several possible epithelial targets, albeit a major one, and IgG envoplakin antibody levels correlate with extent of mucocutaneous disease in patients with PNP/PAMS.