Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Catastrophic antiphospholipid syndrome (CAPS) is a rare condition characterized by multiple thromboses affecting mainly small vessels in a short period of time in patients with antiphospholipid antibodies. A high suspicion index is mandatory in order to initiate rapidly aggressive immunomodulatory therapy to avoid a very poor prognosis. Systemic lupus erythematosus (SLE) is often associated with antiphospholipid syndrome, with a worse outcome when the catastrophic features occur. We report the case of a 64-year-old woman with a clinical debut of SLE who presented concomitantly with CAPS with several thrombosis affecting the kidney, spleen and bilateral limbs with blue toe syndrome in both legs. Furthermore, she presented with aortitis, with a malaise and myalgias and general syndrome (asthenia, hyporexia and mild weight loss). Fortunately, she had a good response to multi-target combination therapy (anticoagulants, corticosteroids, hydroxychloroquine, intravenous immunoglobulins, plasma exchange and rituximab). Here, we discuss the association between aortitis and CAPS secondary to SLE, and review the literature regarding similar conditions.
Lupus 2020 Aug
PMID:Aortitis in the setting of catastrophic antiphospholipid syndrome in a patient with systemic lupus erythematosus. 3251 72

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by heterogeneous symptoms that can manifest in any organ, and often presents at a young age. Infectious mononucleosis (IM) is the acute clinical manifestation of Epstein-Barr virus (EBV). It is characterized by low-grade fever, malaise, lymphadenopathy, splenomegaly, and occasionally symmetrical arthralgias. It has been proposed that EBV is a trigger for new-onset SLE, and patients with autoimmune disorders such as SLE are more likely to have recurrent IM infections. The patient, a 64-year old Caucasian female who's only past medical history was hypertension, developed several months-long period of vague symptoms, including fatigue, malaise, nausea, and nonbilious vomiting with oral intake. She presented with symmetrical polyarthritis involving the hands and elbows, with no history of arthritis before this episode. At the 5-month follow-up, she presented with worsening arthritis bilaterally in her elbows and in her right knee. For several decades, there has been a theoretical association between EBV and SLE, with EBV thought to be one of the many possible triggers for development of SLE. Based on the disease course, we theorize that the patient's IM and EBV infection led to development of SLE. A small fraction of SLE cases have been reported in literature to be associated with EBV. This case adds to that literature with EBV triggering development of SLE in a seemingly previously asymptomatic patient.
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PMID:Development of Systemic Lupus Erythematosus After Infectious Mononucleosis in a 64-Year-Old Woman. 3296 55


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