Subcutaneous Edema, Distal Weakness and Dysphagia Associated with the NXP2 Antibody

Jemima Albayda¹, Iago Pinal-Fernandez², Lisa Christopher-Stine³, Sonye K. Danoff⁴, Cheilonda Johnson⁵, Christopher Mecoli⁶, Julie J. Paik⁷, Alim Ramji⁸ and Andrew Mammen⁹, ¹Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, ²Autoimmune Systemic Diseases Unit, Vall D’Hebron University Hospital, Universitat Autònoma de Barcelona, Barcelona, Spain, ³Ste 4100 Rm 409, Johns Hopkins University School of Medicine, Baltimore, MD, ⁴Medicine/Pulmonary, Johns Hopkins School of Medicine, Baltimore, MD, ⁵Medicine/Pulmonology, Johns Hopkins University, Baltimore, MD, ⁶Rheumatology, Johns Hopkins University, Baltimore, MD, ⁷Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, ⁸School of Medicine, Johns Hopkins University, Baltimore, MD, ⁹Center Tower Ste 5300, Johns Hopkins University School of Medicine, Baltimore, MD

Meeting: 2015 ACR/ARHP Annual Meeting

Date of first publication: September 29, 2015

Keywords: Antibodies and myositis

Session Information

Date: Sunday, November 8, 2015

Title: Muscle Biology, Myositis and Myopathies Poster

Session Type: ACR Poster Session A

Session Time: 9:00AM-11:00AM

Background/Purpose:

Myositis specific antibodies are known to be associated with specific phenotypes. The unusual presentation of subcutaneous edema, distal weakness and dysphagia was seen in several successive patients who were found to be positive for the NXP2 antibody. Further analysis was done to determine if this association was more common with this antibody.

Methods:

A retrospective chart review was completed on patients seen in the Johns Hopkins Myositis Center during the period of 2002-2015. Patients were included in our analysis if they met Bohan and Peter criteria for definite or probable dermatomyositis, Sontheimer’s criteria for amyopathic dermatomyositis, or dermatomyositis by muscle biopsy using ENMC criteria, with known antibody status and available clinical data. Patients who had confirmed autoantibody testing at a CLIA certified lab were included in this study. One hundred and twenty two patients met the criteria and were included for analysis, with 40 patients being NXP2 positive. Subcutaneous edema was defined as pitting or non-pitting edema of one or more extremity that accompanied the active phase of the disease. Muscle strength was graded for 16 muscle groups using the Medical Research Council (MRC) scale. Dysphagia was determined clinically by patient report. Parametric analysis was used to determine the difference between these variables in patients positive for the NXP2 antibody and those with antibodies other than NXP2.

Results:

The edematous phenotype was seen more commonly in the NXP2 + patients than in the NXP2- patients (45% vs. 10%, p<0.001). Overall, NXP2 + patients had statistically significant weakness in distal muscle groups (finger extensors, ankle flexors and ankle extensors) as compared to NXP2- patients. NXP2 is also associated with dysphagia (68% in NXP2+ vs 31% in NXP2-, p<0.001). In NXP2+ patients with edema, there was an associated increase in weakness (significant at the deltoids, triceps, wrist extensors and ankle extensors). There was also a trend to have more dysphagia than in those without edema (82% in those with edema vs 57% in those without it, p=0.1).

Conclusion:

A specific phenotype of subcutaneous edema associated with more severe muscle weakness, distal involvement and dysphagia is associated with the NXP2 antibody.

Disclosure: J. Albayda, None; I. Pinal-Fernandez, None; L. Christopher-Stine, None; S. K. Danoff, None; C. Johnson, None; C. Mecoli, None; J. J. Paik, None; A. Ramji, None; A. Mammen, None.

To cite this abstract in AMA style:

Albayda J, Pinal-Fernandez I, Christopher-Stine L, Danoff SK, Johnson C, Mecoli C, Paik JJ, Ramji A, Mammen A. Subcutaneous Edema, Distal Weakness and Dysphagia Associated with the NXP2 Antibody [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/subcutaneous-edema-distal-weakness-and-dysphagia-associated-with-the-nxp2-antibody/. Accessed .

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