ABSTRACT
POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes) is a rare paraneoplastic disorder due to an underlying plasma cell dyscrasia. The diagnosis of POEMS syndrome requires a chronic polyneuropathy and a monoclonal lambda plasma cell-proliferative disorder (mandatory criteria), and various systematic symptoms such as sclerotic bone lesions, Castleman's disease, organomegaly, endocrinopathy, skin changes, papilloedema and biological abnormalities such as elevated vascular endothelial growth factor (VEGF), thrombocytosis or polycythaemia. We describe an observation of a patient with recurrent thrombosis with thrombocytosis that, after excluding a myeloproliferative neoplasm, proved to be due to POEMS syndrome. This case is unusual compared to the foreground thrombotic symptomatology. POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes) is a rare multi-systematic paraneoplastic disorder due to an underlying plasma cell disorder. The diagnosis of POEMS syndrome requires the presence of both mandatory criteria (a chronic polyneuropathy and a monoclonal plasma cell-proliferative disorder, always lambda restricted); at least one major (among sclerotic bone lesions, Castleman's disease, elevated VEGF (vascular endothelial growth factor)) and one minor criterion (among organomegaly, endocrinopathy, skin changes (haemangiomas, hypertrichosis, hyperpigmentation), papilloedema and thrombocytosis or polycythaemia. We describe an unusual observation of a young patient with recurrent thrombosis with thrombocytosis that, after excluding a myeloproliferative neoplasm, proved to be due to POEMS syndrome.
Subject(s)
POEMS Syndrome/diagnosis , Thrombosis/diagnosis , Adrenal Gland Diseases/diagnosis , Adrenal Gland Diseases/etiology , Adrenal Glands/blood supply , Adult , Diagnosis, Differential , Humans , Infarction/diagnosis , Infarction/etiology , Male , POEMS Syndrome/complications , Thrombosis/etiologyABSTRACT
BACKGROUND: Sarcoidosis is one of the leading causes of uveitis. To date, no studies have assessed the factors specifically related with recovery in ocular sarcoidosis. In this study, we aimed to determine factors associated with ocular and extraocular recovery in patients with sarcoid uveitis. METHODS: A retrospective study of sarcoid uveitis, with a three-year minimum follow-up in Lyon University Hospital between December 2003 and December 2019. Patients presented biopsy-proven sarcoidosis or presumed sarcoid. Recovery was defined by a disease-free status, spontaneously or despite being off all treatments for three years or more. RESULTS: 143 patients were included: 110 with biopsy-proven and 33 with presumed sarcoid uveitis. Seventy-one percent were women, the median age at presentation was 53 years, and 71% were Caucasian. Chronic uveitis was the main clinical presentation (75%), mostly panuveitis (48%) with bilateral involvement (82%). After a median follow-up of 83.5 months, recovery was reported in 26% of patients. In multivariable analysis, Caucasian ethnicity (p = 0.007) and anterior uveitis (p = 0.008) were significantly associated with recovery, while increased intraocular pressure was negatively associated (p = 0.039). CONCLUSION: In this large European cohort, one quarter of patients recovered. Caucasian ethnicity and anterior uveitis are associated with ocular and extraocular recovery.