Introduction: Treatment of neurosarcoidosis is based on case and cohort studies and expert opinion. Based on this limited evidence, tumor necrosis factor alpha (TNF) inhibitors are used in treatment. TNF inhibitors have been shown to induce autoantibodies in some patients, including anti-nuclear (ANA) and anti-double-stranded DNA (dsDNA) antibodies. Some patients further develop a clinical lupus syndrome.
Clinical Case Description: We present a 59-year-old woman with lymph node biopsy-confirmed sarcoidosis and probable neurosarcoidosis.
She initially noted hand and leg paresthesias and cramping. Imaging revealed leptomeningeal enhancement around the brainstem and infundibulum and enhancement of the distal cord, conus, cauda equina, and nerve root sleeves. Lumbar puncture revealed lymphocytosis, elevated protein, hypoglycorrhachia and no oligoclonal bands. Flow cytometry of CSF revealed CD4+ T cell predominance. FDG-PET revealed avidity of the spleen, skeleton, and innumerable lymph nodes. Cervical node biopsy revealed granulomatous lymphadenitis and sarcoidosis was diagnosed.
She later developed severe headache, vomiting, polyuria, and thirst. MRI showed new hypothalamic enhancement. Central diabetes insipidus was diagnosed. She was given a methylprednisolone pulse, a prednisone taper, and the TNF inhibitor infliximab, with radiographic and clinical improvement.
She later developed pleuritic chest pain, fever, tachycardia, and inflammatory arthritis. ANA and dsDNA testing were newly positive. Infliximab was stopped, oral steroids were intensified, and colchicine and hydroxychloroquine were started. Tocilizumab was selected as next line of therapy.
Conclusions: Anti-TNF induced lupus is a rare but potentially serious effect of anti-TNF therapy, particularly infliximab. Its features include frequent presence of dsDNA antibodies and greater potential for organ threat, unlike classical drug-induced lupus. In patients with sarcoidosis, it represents a challenge in further therapeutic selection. Optimal patterns of surveillance and indications for termination of TNF therapy have not been defined. Clinical suspicion should be high in patients on TNF inhibitors who have new symptoms consistent with SLE.