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Hemophagocytic lymphohistiocytosis (HLH) is a rare and potentially fatal hyperinflammatory syndrome characterized by dysregulated immune activation and systemic inflammation. Secondary HLH is often triggered by infections, with being an infrequently reported cause. Peripheral axonal neuropathy is a rare and poorly understood complication of HLH.

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Article Synopsis
  • There are various inflammatory myopathies, including juvenile dermatomyositis (JDM), which can cause muscle inflammation and weakness, with macrophage activation syndrome (MAS) being a severe complication, though it is not frequently reported.
  • A case study of a six-year-old girl with proximal muscle weakness and skin manifestations, leading to a JDM diagnosis, showed elevated lab markers and confirmed muscle involvement through MRI.
  • The girl initially improved with steroid therapy, but later tested positive for anti-MDA5 antibodies, indicating a worse prognosis and highlighting the importance of recognizing MAS as a possible complication in JDM patients with these antibodies.
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Adult-onset Still's disease (AOSD) is an uncommon systemic inflammatory disorder that presents with diverse, overlapping symptoms, complicating the diagnostic process due to its nonspecific clinical features and the absence of a definitive diagnostic test. Diagnosis is often challenging and relies on excluding other conditions while maintaining a high index of suspicion, supported by specific diagnostic criteria such as Yamaguchi or Fautrel. Prompt recognition and a multidisciplinary approach are essential, as AOSD can progress to life-threatening multiorgan dysfunction due to a hyperinflammatory response.

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Article Synopsis
  • - Adult-onset Still's disease (AOSD) is a rare, inflammatory condition that typically presents with fever, joint pain, and a distinctive rash, and is not well understood.
  • - A unique case is described involving a 37-year-old man whose AOSD was triggered by a throat infection caused by Group A streptococci (GAS), leading to severe inflammation and fulfilling diagnostic criteria for AOSD.
  • - The patient was treated successfully with NSAIDs and steroids, resulting in significant improvement, suggesting that GAS infections can potentially be a trigger for AOSD, emphasizing the importance of exploring less common causes of the disease.
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Ferritin is commonly used as a marker for iron status, aiding in diagnosing iron deficiency anemia. However, it is also an acute phase reactant often elevated in various inflammatory conditions. Marked hyperferritinemia, defined as ferritin levels above 10,000 μg/L, can indicate severe underlying conditions, including infections, cardiovascular like heart failure, endocrinological, autoimmune, and malignancies.

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