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Sudden cardiac death caused by Kawasaki coronary artery vasculitis in a child with Hodgkin's lymphoma. Case report and literature review.
Cardiovasc Pathol
November 2024
Department of Precision and Regenerative Medicine and Jonian Area, Pathology Unit, University of Bari, Bari, Italy.
Article Synopsis
- Coronary artery vasculitis is a rare illness that mainly affects the heart's blood vessels and can happen because of conditions like Kawasaki Disease.
- A 6-year-old boy with cancer died suddenly because of a blood clot in his heart caused by this vasculitis, likely linked to Kawasaki Disease.
- Researchers are studying the features of Kawasaki Disease by looking at many studies from the past to learn more about how it affects people of all ages.
Top 10 Clinical Pearls in Vasculitic Neuropathies.
Semin Neurol
February 2025
Department of Neurology, Virginia Commonwealth University, Richmond, Virginia.
Article Synopsis
- Vasculitic neuropathies are inflammatory conditions affecting the peripheral nerves, often caused by systemic vasculitis or related disorders, leading to painful sensory and motor deficits.
- Diagnosis involves extensive tests, including lab work, electrodiagnostics, and tissue biopsies, while treatment typically includes immunosuppressants and pain management.
- Timely recognition and intervention are crucial to prevent serious complications, including permanent nerve damage or fatal outcomes.
[Vasculitic involvement of the skeletal muscle and the peripheral nervous system: clinical and neuropathologic perspective].
Z Rheumatol
September 2024
Institut für Neuropathologie, Charité - Universitätsmedizin, Berlin, Deutschland.
Article Synopsis
- The peripheral nervous system and skeletal muscle are commonly affected in systemic vasculitis, leading to symptoms like myalgia, muscle weakness, and sensory deficits.
- Vasculitic myopathy (VM) is marked by severe muscle pain but normal creatine kinase levels, and can be identified by MRI, while vasculitic neuropathy (VN) causes motor and sensory deficits, especially in the lower limbs.
- Diagnosing these conditions typically involves histopathological examination of nerve and muscle biopsies, which is crucial for determining treatment strategies.
Clinical spectrum and outcome of children with deficiency of adenosine deaminase 2 (DADA2): multicentric experience from India.
Rheumatology (Oxford)
September 2024
Department of Pediatric Rheumatology, Bristol Royal Hospital for Children, Bristol, UK.
Article Synopsis
- DADA2 is a genetic disorder similar to polyarteritis nodosa that affects children, and this study focuses on describing its clinical presentation and outcomes among Indian patients.* -
- A total of 16 children with DADA2 were studied, showing common symptoms like fever and rash, with over half experiencing CNS strokes; they were treated with immunomodulators like steroids and anti-TNF agents.* -
- Most children responded positively to treatment, achieving remission, and the study highlights key differences between DADA2 and classic polyarteritis nodosa, especially in terms of age of onset and CNS involvement.*
Sight-Threatening Unilateral Posterior Scleritis With Positive Atypical p-ANCA As Early Manifestation of Lupus Spectrum Disease.
Cureus
April 2024
Department of Ophthalmology, Hospital Ampang, Selangor, MYS.
Antinuclear cytoplasmic antibody (ANCA)-related scleritis is a potentially sight-threatening inflammatory condition that may occur as a primary vasculitis disorder or as a secondary vasculitis in a variety of inflammatory conditions. While ANCA has been classically associated with primary vasculitis diseases such as granulomatosis with polyangiitis (GPA), microscopic polyarteritis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA), it is interesting that in cases of lupus spectrum disease (LSD), both ANCA and atypical p-ANCA have been observed as secondary autoantibodies. Scleritis is a rare ocular manifestation of lupus disease with an incidence of around 1%.
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