Background The neuropsychiatric lupus erythematosus (NPSLE) is a severe complication of systemic lupus erythematosus (SLE) that is characterized by a variety of neurological manifestations involving both central and peripheral nervous system with variable mechanisms. This study aimed to investigate the frequency of NPSLE and its relation to other clinical and laboratory findings in SLE patients. Results We retrieved the medical records of 134 SLE patients during the study period; of them, 68 patients (50.7%) had NPSLE. Headache (55. 9%) was the most frequent NPSLE manifestation followed by seizures (54.4%), psychosis was the third most frequent one with a percentage of 41.2. The demographic data didn’t differ in patients with and without NPSLE. NPSLE patients had lower complement 3 (C3) (p = 0.025) and C4 (p = 0.008) levels, more lupus anticoagulant level (p = 0.033) and more frequency of antiphospholipid syndrome (p = 0.030). There was no statistical difference regarding the drug intake or other laboratory findings. Disease activity and damage indices didn’t differ in both groups. Conclusion The prevalence of NPSLE in this study was 50.7%. Headache, seizures and psychosis were the most frequent neuropsychiatric manifestations in the studied patients. SLE patients with neuropsychiatric manifestations had lower complement levels, higher lupus anticoagulant antibodies and antiphospholipid syndrome.
Background Familial Mediterranean Fever (FMF) is a periodic auto-inflammatory disease with multiple systemic manifestations. This study aims to describe the various musculoskeletal and neurological manifestations in a cohort of Egyptian FMF patients and to evaluate their relation to the different Mediterranean fever gene (MEFV) mutations. Results This study involved 145 FMF patients, of them 62.1% were females and 31.7% were of the pediatric age. All involved patients had homozygous MEFV gene mutation. The presenting manifestation in 71.9% of these patients was abdominal pain followed by musculoskeletal manifestations in 35.2% of them. 38.6 % of the involved patients had arthritis during the period of follow-up. Monoarthritis was the most frequent pattern of arthritis. Arthralgia was present in 96.6% of the studied patients. Myalgia was present in 19.3% of the studied patients especially involving the lower limb muscles with one case of protracted febrile myalgia. Neurological manifestations were present in about 86.9 % of patients with vertigo, paresthesia, and seizures as the most common. Five major MEFV gene mutations were found in most of the studied patients (135 patients): M694V, M680I, E148Q, V726A, and M694I. When a comparative study was done between these five major mutations according to the age of onset of the symptoms, different musculoskeletal and neurological manifestations, ESR, serum amyloid level and dose of colchicine, no statistical difference was found. Conclusion Musculoskeletal manifestation is the second most common presenting symptom in a cohort of Egyptian FMF patients after abdominal pain. Arthralgia is the most frequent musculoskeletal manifestation while monoarthritis of the knee or ankle joint is the most common pattern of arthritis in FMF patients. Vertigo, paresthesia, and seizures are the most frequent neurological manifestations. Musculoskeletal manifestations, neurological manifestations, serum amyloid level, and dose of colchicine are not related to the type of the genetic mutation in this cohort.
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