Abstract
IntroductionSarcoidosis is a multisystem disease characterized by noncaseating granulomas in the lungs, skin, lymph nodes, and, rarely, the nervous system. Granuloma formation in sarcoidosis is mediated by increased secretion of interferon-gamma, interleukin-2, and tumor necrosis factor-alpha. 25% of patients with neurosarcoidosis are steroid resistant and another 20–40% are resistant to any conventional immunosuppression, but the typical agents suppress the immune system in a non-specific fashion. Thalidomide has been shown to have activity specific to the inflammatory mediators of sarcoidosis, has been shown to be beneficial in cutaneous sarcoidosis, and provides an interesting observation in our patient with refractory neurosarcoidosis.Case presentationA 40 year old African-american female presented with refractory neurosarcoidosis. Over the course of several years, the patient was treated with high dose steroids, imuran, cytoxan, and cyclosporine without benefit. Then, the patient received thalidomide, slowly escalating to 650 mg. After 2 months radiologic improvement was noted and after 6 months clinical stabilization and improvement became apparent.ConclusionOur case report presents a difficult, refractory case of neurosarcoidosis that demonstrates an altered prognosis based on the addition of thalidomide.
Highlights
Sarcoidosis is a multisystem disease characterized by noncaseating granulomas in the lungs, skin, lymph nodes, and, rarely, the nervous system
Our case report presents a difficult, refractory case of neurosarcoidosis that demonstrates an altered prognosis based on the addition of thalidomide
25% will still have a refractory course with steroid treatment and, even more concerning, 20–40% of those refractory patients will not respond to any level of current conventional immunosuppression [1,3]
Summary
Sarcoidosis is a granulomatous, inflammatory disease that most commonly involves the lungs, skin, and eyes, but can affect the nervous system in about 5% of cases [1]. The avonex was discontinued, and the patient was started on imuran (50–100 mg/day), while continuing prednisone (60–80 mg po QOD) over the 6 months. MsbFhiilRgoatIuwefrirneagdl1iomnpgastrickoenderemvneohsna(nt6hcembmemfeo)nrteoinf itniafutinodniboufluthmal(id6ommmid)ea, nd MRI findings one month before initiation of thalidomide, showing marked enhancement of infundibulum (6 mm) and bilateral optic nerves (6 mm). After two months of treatment, the patient had radiographic improvement on follow-up MRI scan (see figure 2). Following another 4 to 10 months of thalidomide, there was complete resolution of all abnormal enhancement on MRI, which has been maintained over the past several years (see figure 3). Cyclosporine was discontinued and prednisone was slowly weaned to 5 mg per day Throughout this wean, the previously noted clinical improvements remained stable.
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