Overlapping features of polymyositis and inclusion body myositis in HIV-infected patients

Thomas Lloyd, Iago Pinal-Fernandez, Elizabeth Michelle, Lisa Christopher-Stine, Katherine Pak, Ned Sacktor, Andrew L. Mammen

Research output: Contribution to journalArticle

Abstract

Objective: To characterize patients with myositis with HIV infection. Methods: All HIV-positive patients with myositis seen at the Johns Hopkins Myositis Center from 2003 to 2013 were included in this case series. Muscle biopsy features, weakness pattern, serum creatine kinase (CK) level, and anti-nucleotidase 1A (NT5C1A) status of HIV-positive patients with myositis were assessed. Results: Eleven of 1,562 (0.7%) patients with myositis were HIV-positive. Myositis was the presenting feature of HIV infection in 3 patients. Eight of 11 patients had weakness onset at age 45 years or less. The mean time from the onset of weakness to the diagnosis of myositis was 3.6 years (SD 3.2 years). The mean of the highest measured CK levels was 2,796 IU/L (SD 1,592 IU/L). On muscle biopsy, 9 of 10 (90%) had endomysial inflammation, 7 of 10 (70%) had rimmed vacuoles, and none had perifascicular atrophy. Seven of 11 (64%) patients were anti-NT5C1A-positive. Upon presentation, all had proximal and distal weakness. Five of 6 (83%) patients followed 1 year or longer on immunosuppressive therapy had improved proximal muscle strength. However, each eventually developed weakness primarily affecting wrist flexors, finger flexors, knee extensors, or ankle dorsiflexors. Conclusions: HIV-positive patients with myositis may present with some characteristic polymyositis features including young age at onset, very high CK levels, or proximal weakness that improves with treatment. However, all HIV-positive patients with myositis eventually develop features most consistent with inclusion body myositis, including finger and wrist flexor weakness, rimmed vacuoles on biopsy, or anti-NT5C1A autoantibodies.

Original languageEnglish (US)
Pages (from-to)1454-1460
Number of pages7
JournalNeurology
Volume88
Issue number15
DOIs
StatePublished - Apr 11 2017

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Inclusion Body Myositis
Polymyositis
Myositis
HIV
Creatine Kinase
nucleotidase
Vacuoles
Wrist
Biopsy
Age of Onset
Fingers
HIV Infections
Muscles
Muscle Strength
Immunosuppressive Agents
Ankle
Autoantibodies
Atrophy
Knee

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Overlapping features of polymyositis and inclusion body myositis in HIV-infected patients. / Lloyd, Thomas; Pinal-Fernandez, Iago; Michelle, Elizabeth; Christopher-Stine, Lisa; Pak, Katherine; Sacktor, Ned; Mammen, Andrew L.

In: Neurology, Vol. 88, No. 15, 11.04.2017, p. 1454-1460.

Research output: Contribution to journalArticle

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abstract = "Objective: To characterize patients with myositis with HIV infection. Methods: All HIV-positive patients with myositis seen at the Johns Hopkins Myositis Center from 2003 to 2013 were included in this case series. Muscle biopsy features, weakness pattern, serum creatine kinase (CK) level, and anti-nucleotidase 1A (NT5C1A) status of HIV-positive patients with myositis were assessed. Results: Eleven of 1,562 (0.7{\%}) patients with myositis were HIV-positive. Myositis was the presenting feature of HIV infection in 3 patients. Eight of 11 patients had weakness onset at age 45 years or less. The mean time from the onset of weakness to the diagnosis of myositis was 3.6 years (SD 3.2 years). The mean of the highest measured CK levels was 2,796 IU/L (SD 1,592 IU/L). On muscle biopsy, 9 of 10 (90{\%}) had endomysial inflammation, 7 of 10 (70{\%}) had rimmed vacuoles, and none had perifascicular atrophy. Seven of 11 (64{\%}) patients were anti-NT5C1A-positive. Upon presentation, all had proximal and distal weakness. Five of 6 (83{\%}) patients followed 1 year or longer on immunosuppressive therapy had improved proximal muscle strength. However, each eventually developed weakness primarily affecting wrist flexors, finger flexors, knee extensors, or ankle dorsiflexors. Conclusions: HIV-positive patients with myositis may present with some characteristic polymyositis features including young age at onset, very high CK levels, or proximal weakness that improves with treatment. However, all HIV-positive patients with myositis eventually develop features most consistent with inclusion body myositis, including finger and wrist flexor weakness, rimmed vacuoles on biopsy, or anti-NT5C1A autoantibodies.",
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