Cutaneous involvement in anti-HMGCR positive necrotizing myopathy.

Williams, Barbara; Horn, Michael P.; Banz, Yara; Feldmeyer, Laurence; Villiger, Peter M. (2021). Cutaneous involvement in anti-HMGCR positive necrotizing myopathy. Journal of autoimmunity, 123(102691), p. 102691. Elsevier 10.1016/j.jaut.2021.102691

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OBJECTIVE

Anti-3-Hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) positive immune-mediated necrotizing myopathy (IMNM) is a rare disease. It is induced by exogenous substances, most often by statins. Little is known about cutaneous manifestations of HMGCR positive IMNM and about HMGCR antibody positivity in other diseases.

METHODS

The characteristics of patients with anti-HMGCR autoantibodies measured at our laboratory between January 2012 and September 2020 were studied. Characteristics of patients with IMNM were compared to those patients with positive antibodies but without muscle involvement. Associations of IMNM with other organ involvements were searched for.

RESULTS

Of the 32 patients studied, 23 showed characteristics of IMNM, 9 did not fulfill current classification criteria but most showed signs of connective tissue diseases. Patients with IMNM were older (66 and 35 years, respectively; 0.92 (0.73-0.98); p < 0.001), had more frequent statin exposure (87% and 33%, respectively; 0.84 (0.61-0.94); p = 0.005) and higher mean peak CK (8717U/l and 329U/l, respectively; 1.0 (0.85-1.0); p < 0.001). 13/23 (56%) of IMNM patients showed cutaneous lesions; none of the patients suffered from cancer; only three IMNM patients showed drug-free complete remission. Incidence of IMNM in the catchment area of our center is at least 2.7/Mio/year.

CONCLUSION

Cutaneous lesions were found to be more frequent in anti-HMRCR positive IMNM than previously reported. Titer of anti-HMGCR antibodies and CK levels were significantly higher in IMNM than in other autoimmune connective tissue diseases. The data support the hypothesis of an antigen-driven response in IMNM, and suggests an activation of autoreactive B-lymphocytes in non-IMNM patients.

Item Type:

 Journal Article (Original Article)

Division/Institute:

 04 Faculty of Medicine > Department of Haematology, Oncology, Infectious Diseases, Laboratory Medicine and Hospital Pharmacy (DOLS) > Institute of Clinical Chemistry
04 Faculty of Medicine > Department of Dermatology, Urology, Rheumatology, Nephrology, Osteoporosis (DURN) > Clinic of Rheumatology and Immunology
04 Faculty of Medicine > Department of Dermatology, Urology, Rheumatology, Nephrology, Osteoporosis (DURN) > Clinic of Dermatology
04 Faculty of Medicine > Service Sector > Institute of Pathology > Clinical Pathology

UniBE Contributor:

 Horn, Michael (B), Banz Wälti, Yara Sarah, Feldmeyer, Laurence, Villiger, Peter Matthias

Subjects:

 600 Technology > 610 Medicine & health

ISSN:

 0896-8411

Publisher:

 Elsevier

Language:

 English

Submitter:

 Karin Balmer

Date Deposited:

 20 Aug 2021 08:59

Last Modified:

 29 Mar 2023 23:37

Publisher DOI:

 10.1016/j.jaut.2021.102691

PubMed ID:

 34332436

Uncontrolled Keywords:

 Connective tissue disease HMGCR Immune-mediated necrotizing myopathy (IMNM) Myositis Necrotizing autoimmune myopathy (NAM) Necrotizing myopathy Statin-induced myopathy

BORIS DOI:

 10.48350/157901

URI:

 https://boris.unibe.ch/id/eprint/157901

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