E033 The evolution of neurosarcoidosis on infliximab: a case report
Authors: Jayasekera Hirushi S , Tabassum Syeda A , Douglas Karen M J 
Published: April 2023

Background/Aims

We report a case of neurosarcoidosis (NS) 16 years after initial diagnosis of systemic sarcoidosis in a patient treated with methotrexate, infliximab and corticosteroids.

Methods

In 2015 a 47-year-old male presented with florid synovitis in MCPs, PIPs, in a pattern reminiscent of rheumatoid arthritis (rheumatoid factor, anti-CCP antibodies were negative; CRP 46g/l). Previously in 2006 a diagnosis of sarcoidosis had been made due to systemic symptoms, arthralgia, bilateral hilar lymphadenopathy (BHL) and confirmed on biopsy of bone and mediastinal nodes, for which he was currently prescribed prednisolone 10mg. So in 2015, considering this to be an inflammatory arthritis (IA), in the presence of sarcoid, methotrexate 15mg subcutaneously was added with good response initially. Though his BHL had regressed and he has was systemically well his IA subsequently flared, and after liaising with his respiratory consultant, adalimumab was added in 2017. Subsequently, as he was well, prednisolone was weaned to 3mg daily. In May 2020 his IA flared again, this time with DIP involvement and dactylitis (his father has psoriasis). Adalimumab was switched to baricitinib, and prednisolone increased to 15mg daily. Baricitinib was inefficacious and linked to recurrent sinusitis so this was switched to infliximab in January 2022 and methotrexate increased to 25mg weekly with resolution of his IA. The patient presented in September 2022 with confusion, slurred speech and unsteady gait with a background of recurrent headaches for six months since starting infliximab.

Results

Routine blood tests were normal: full blood count, biochemistry, serum ACE, calcium and CRP 2mg/L. Chest X-ray was normal. Neuroimaging: MRI (non-contrast) and CT head were normal. CSF fluid: protein 1.75(0.15-0.4)g/l; glucose 1.8(2.2-3.9)mmol/L; Lymphocytes 98cmm; bacterial and viral culture negative. A diagnosis of neurosarcoidosis was made. Prednisolone was increased to 30mg daily with resolution of neurological symptoms and headache.

Conclusion

In the UK the incidence of NS is 1/100,000. NS is reported in 5-10% of patients with sarcoidosis and typically presents within 2 years of diagnosis. Presentation of NS includes: cranial neuropathies (50-75%), aseptic meningitis (10-20%), parenchymal disease (&lt;50%), hydrocephalus (10%), sellar disease (2-8%), myopathy (10%), myelopathy (5-26%), neuropsychiatric illness (depression 60%, psychosis 20%) and peripheral neuropathy (2-86%). Typical CSF examinations in NS are: a mild to moderate pleocytosis (usually &lt;100cells/μL), with lymphocyte predominance and elevated protein. Though Infliximab is reported as a treatment option for NS, we reflect that anti-TNF alpha therapies, though they reduce systemic inflammation, do not cross the blood brain barrier. We speculate that introduction of infliximab unmasked NS in this patient.

This case also highlights the importance of probing and investigating neurological symptoms in patients with sarcoidosis.

Disclosure

H.S. Jayasekera: None. S.A. Tabassum: None. K.M.J. Douglas: None.