Paraneoplastic Pemphigus (Paraneoplastic Autoimmune Multiorgan Syndrome) Screening Antibodies by IIF

Casandra

Casandra Test Code AR23279Version 1 (DRAFT)

Performing Lab

Lab ARUP LaboratoriesLab Test ID 0092107CPT Codes 88346, 88350

Paraneoplastic Pemphigus (Paraneoplastic Autoimmune Multiorgan Syndrome) Screening Antibodies by IIF

Also known as: PARA PEMPH

Clinical Use

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Use

Paraneoplastic pemphigus (PNP), also known as paraneoplastic autoimmune multiorgan syndrome (PAMS), is a severe mucocutaneous blistering and erosive disease associated with underlying malignancies, most often hematologic in nature, such as lymphoma and leukemia, and sarcoma. It affects individuals of all ages and can also occur in association with benign neoplasms, like Castleman disease, which is more frequent in children and adolescents. The presence of antibodies targeting various epithelial components can lead to the involvement of multiple organs, such as the eyes, lungs, and gastrointestinal tract, which forms the basis of the multiorgan syndrome. This test is intended to screen for IgG antibodies associated with paraneoplastic pemphigus using indirect immunofluorescence (IIF) on rodent substrates.

Special Instructions

This test is not the preferred initial test for paraneoplastic pemphigus. The preferred testing includes an expanded panel with ELISA for IgG envoplakin antibodies and indirect immunofluorescence on rat bladder and other rodent substrates. The test may be used if the IgG envoplakin antibody ELISA has been performed separately and for the monitoring of antibodies in patients with paraneoplastic pemphigus who do not have increased IgG envoplakin antibodies. Correlation with direct immunofluorescence, tissue biopsy, and other epithelial antibody assessments is recommended.

Limitations

This screening includes only indirect immunofluorescence with rodent substrates and does not provide IgG envoplakin antibody ELISA testing. It is important to note that negative results do not rule out PNP/PAMS or other malignancy-associated disorders. Results should be correlated with direct immunofluorescence on tissue biopsy, histopathological examination, and additional serum antibody testing including IgG envoplakin by ELISA for a comprehensive clinical assessment. The indirect immunofluorescence method offers broader detection than ELISA, which may be more sensitive for low-level antibodies.

Test Details

New York Approved