Paraneoplastic Pemphigus (Paraneoplastic Autoimmune Multiorgan Syndrome) Antibody Screening Panel
Use to assess and monitor paraneoplastic pemphigus, a rare paraneoplastic disease associated with lymphoproliferative disorders/malignancies and demonstrating clinical features of severe pemphigus. Testing should be correlated with concurrent Direct Immunofluorescence, Tissue Biopsy (Cutaneous, Mucosal, Epithelial) (0092572), histopathological examination of formalin-fixed tissue, and assessment of other epithelial antibodies.
Consider ordering concurrently with Immunobullous Disease Antibody Panel (3001409) for broad epithelial antibody assessment. Alternatively, order with Desmoglein 1 and Desmoglein 3 (Pemphigus) Antibodies, IgG by ELISA (0090649) and Bullous Pemphigoid (BP180 and BP230) Antibodies, IgG by ELISA (0092566) because any of these epithelial antibodies may be increased in paraneoplastic pemphigus.
If other more common types of pemphigus are diagnostic considerations, order Pemphigus Antibody Panel, IgG (0090650) with or without the rarer IgA pemphigus variant test, Pemphigus Antibodies, IgA by IIF (0092106), concurrently with this test.
For more information about components, methodology, and specimen type, see the ARUP Immunobullous Disease Testing Comparison tool.
Semi-Quantitative Indirect Immunofluorescence (IIF)
New York DOH Approval Status
Plain red or serum separator tube (SST).
Transfer 2 mL serum to an ARUP Standard Transport Tube. (Min: 0.5 mL)
Hemolyzed or lipemic specimens. Plasma.
Ambient: 1 week; Refrigerated: 2 weeks; Frozen: Indefinitely
Refer to report
Performed by non-ARUP Laboratory
The methodology is indirect immunofluorescence (IIF) of patient serum on substrates from rodents including rat bladder, mouse bladder, mouse heart, and mouse liver to detect characteristic antibody reactivity: simple columnar epithelial cell surface and basement membrane zone in bladders, intercalated discs in heart, and portal tracts in liver. Monkey esophagus substrate is included if other concurrent IIF testing does not. For specimens less than 0.5 mL, call the Immunodermatology Laboratory at (866) 266-5699.
This test should be distinguished from antibody testing of cerebral spinal fluid (CSF) for paraneoplastic neurologic syndromes; 3004510, 3004512, 3004517 are different tests.
88346; 88350 x4
|Component Test Code*||Component Chart Name||LOINC|
|0092107||Paraneoplastic Pemphigus Ab Screen||42936-5|
- cell surface and basement membrane zone antibodies
- envoplakin antibodies
- intercellular substance
- mixed epithelial antibodies
- paraneoplastic autoimmune multiorgan syndrome (PAMS)
- Paraneoplastic Pemphigus Antibody Screen
- periplakin antibodies
- rat bladder immunofluorescence