A Case of Bilateral Sequential Optic Neuropathies With Pachymeningitis and Aortitis: Difficulty in Differentiating Erdheim-Chester Disease From IgG4-Related Disease
نویسندگان
چکیده
Objective We describe a case of bilateral sequential optic neuropathies with pachymeningitis and aortitis, findings that raised suspicion Erdheim-Chester disease versus IgG-4 related disease. Background (ECD) is rare histiocytic neoplasm characterized by tissue infiltration foamy histiocytes, chronic, uncontrolled inflammation. IgG4-related (IgG4-RD) an insidiously progressive immune-mediated fibrotic typified tumour-like mass formation in many affected organs. Neurologic manifestations are diverse. Design/Methods A 58-year-old male was transferred to our centre for acute onset neuropathies. His visual acuity light perception the right eye 20/50 left eye. Results Enhanced MRI brain orbits showed focal pachymeningeal thickening enhancement anterior cranial fossa over frontal lobe eccentric nerve sheath. CRP elevated (23 mmol/L 62 mmol/L); extensive CSF serum infectious inflammatory investigations were unrevealing. PET body demonstrated aortitis CT angiography suggested coronary artery vasculitis. Bone scan symmetric involvement long bones. Dural biopsy delayed due Covid-19 pandemic completed following protracted steroid course 15 mg/kg dose cyclophosphamide. Pathology mixed infiltrate increased expression IgG4 neutrophils. Clusters CD68+, CD1a, S100-negative macrophages seen all layers dura. No BRAF mutation identified. Conclusions This demonstrates classic imaging ECD including pachymeningitis, bone aortitis. may show characteristic absent this case. challenge interpretation immunosuppressive cytotoxic therapy difficulty differentiating from IgG4-RD.
منابع مشابه
Erdheim-Chester Disease as a Mimic of IgG4-Related Disease
Immunoglobulin-G4 (IgG4)-related disease (IgG4RD) is a fibro-inflammatory disorder characterized by tissue-infiltrating IgG4 plasma cells, and, often, high serum IgG4. Several autoimmune, infectious, or proliferative conditions mimic IgG4RD. Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis, characterized by foamy histiocytic infiltration, fibrosis, and chronic inflammat...
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متن کاملErdheim-Chester Disease
Erdheim-Chester disease (ECD) is a rare, xanthogranulomatous, non-Langerhans cell histiocytosis with frequent systemic involvement. Although the diagnosis is based on characteristic histological and radiological findings, its identification can be challenging because of its heterogeneous presentation. Osteosclerosis of long bones, often associated with bone pain, is the most common initial mani...
متن کاملErdheim-chester disease.
Erdheim-Chester disease (ECD) is a rare, non-Langerhans cell histiocytosis of unknown etiology, characterized by multi-organ involvement. ECD is usually diagnosed on the basis of characteristic radiologic and histopathological findings. Lesions may be skeletal and/or extraskeletal in location, and may include the skin, lung, heart, and central nervous system. We describe here a 68-year-old man ...
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ژورنال
عنوان ژورنال: Neurology
سال: 2022
ISSN: ['0028-3878', '1526-632X']
DOI: https://doi.org/10.1212/01.wnl.0000903476.18885.bc