Affiliations: [a]
Department of Paediatric Neurology, University Hospitals Bristol and Weston NHS Foundation Trust, Bristol, UK
| [b]
Department of Paediatrics, Royal United Hospital, Coombe Park, Bath, UK
| [c]
Department of Neurophysiology, University Hospitals Bristol and Weston NHS Foundation Trust, Bristol, UK
| [d]
Department of Neuropathology, Southmead Hospital, North Bristol Trust NHS, Bristol, UK
| [e]
Paediatric Immunology and Infectious Diseases Service, Bristol Royal Hospital for Children and Bristol Children’s Vaccine Centre, Schools of Cellular and Molecular Medicine Population Health Sciences, University of Bristol, Bristol, UK
Correspondence:
[*]
Correspondence to: Dr Anirban Majumdar, Department of Paediatric Neurology, 6th Floor Education Centre, Upper Maudlin St, Bristol, BS2 8BJ, UK. Tel.: +44 01173420502; E-mail: Anirban.Majumdar@uhbw.nhs.uk.
Abstract: Here, we describe a five year old girl with congenital HIV who had a six-week onset of rapidly deteriorating mobility and progressive proximal muscle weakness, associated with a raised Creatine Kinase (CK) level of 4330 U/L [25–200 U/L], subsequently diagnosed with an inflammatory myositis. Potential causes were investigated by paediatric neurology and immunology teams. Her viral load had been undetectable over the preceding two years, excluding a primary HIV myositis. While MRI scanning did not show evidence of definite myositis, a muscle biopsy showed evidence of an inflammatory process, comprising a moderate endomysial, perimysial and perivascular mononuclear (CD8 + T cell) infiltrate with increased MHC expression. No particular features of dermatomyositis or immune-mediated necrotising myopathy were identified and there were no features of an inclusion body myositis. Given the absence of active HIV infection, the role of anti-retroviral medications was considered. She had had a recent switch in medication, from twice daily Raltegravir (an Integrase Strand Transfer Inhibitor, INSTI) to once daily Dolutegravir (an INSTI) while continuing on an established daily protocol of Abacavir and Lamivudine (Nucleoside Reverse Transcriptase Inhibitors). Changing the Dolutegravir back to Raltegravir, in combination with continuing Lamivudine and Abacavir for two months made no difference to her weakness or CK levels. Moreover, this drug regimen had been well-tolerated over the preceding 19 month period. Changing the anti-retroviral regime completely to a single drug class (Protease Inhibitors) of Ritonavir and Darunavir, resulted in a dramatic improvement in her symptomatology. Within ten days she regained the ability to stand and walk, with a reduction in her CK from 1700 U/L at time of switch to 403 U/L [25–200]. This case highlights the potential risk of developing inflammatory myositis from anti-retrovirals even 19 months into treatment.
