Progressive multifocal leukoencephalopathy in a patient with systemic lupus erythematosus and autoimmune hepatitis.
Saranya GomathyBaikuntha PanigrahiPraveen Kumar TirlangiNaveet WigMegha BrijwalMehar C SharmaAjay GargManjari TripathiSrikant MohtaRamesh DoddamaniDeepti VibhaRajesh Kumar SinghRajni YadavSaumya SahuVaishali SuriKavneet KaurMadhavi TripathiAnshu RohatgiArunmozhimaran ElavarasiPublished in: International journal of rheumatic diseases (2022)
Progressive multifocal leukoencephalopathy (PML) is a rare demyelinating central nervous system illness encountered in the setting of immunosuppressive conditions like human immunodeficiency virus / acquired immunodeficiency syndrome, autoimmune diseases and hematologic malignancies. We had a 54-year-old woman with systemic lupus erythematosus and coexisting autoimmune hepatitis who presented with progressive cognitive decline, right hemiparesis and ataxia who was found to have PML. She had severe CD4 lymphopenia. She was managed with low-dose prednisolone and plasma exchange after which she showed significant clinical improvement. This case highlights the diagnostic and therapeutic challenges encountered in managing a case of PML in the setting of autoimmune conditions with profound lymphopenia.
Keyphrases
- multiple sclerosis
- systemic lupus erythematosus
- human immunodeficiency virus
- cognitive decline
- low dose
- hepatitis c virus
- mild cognitive impairment
- disease activity
- antiretroviral therapy
- case report
- drug induced
- early onset
- hiv infected
- high dose
- hiv aids
- intellectual disability
- rheumatoid arthritis
- cerebrospinal fluid
- nk cells
- upper limb