IgG4-related disease presenting as pachymeningitis in a patient with Systemic Lupus Erythematosus: Case Report
IgG4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory condition that can affect multiple organs except synovial tissue. Common presentations include major salivary and lacrimal gland enlargement, orbital disease, retroperitoneal fibrosis and tubulointerstitial nephritis. Central neurologic manifestations are rare with reports of mostly hypophysitis and hypertrophic pachymeningitis.
Diagnosis is histologic and shows lymphoplasmacytic infiltration with IgG4+ plasma cell proliferation, storiform fibrosis, and obliterative phlebitis. Glucocorticoids form the basis of treatment.
IgG4-related disease presenting as pachymeningitis in a patient with Systemic Lupus Erythematosus: Case Report
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DOI: 10.22533/at.ed.53521040814
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Palavras-chave: IgG4-RD IgG4-related disease, SLE Systemic Lupus Erythematosus
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Keywords: IgG4-RD IgG4-related disease, SLE Systemic Lupus Erythematosus
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Abstract:
IgG4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory condition that can affect multiple organs except synovial tissue. Common presentations include major salivary and lacrimal gland enlargement, orbital disease, retroperitoneal fibrosis and tubulointerstitial nephritis. Central neurologic manifestations are rare with reports of mostly hypophysitis and hypertrophic pachymeningitis.
Diagnosis is histologic and shows lymphoplasmacytic infiltration with IgG4+ plasma cell proliferation, storiform fibrosis, and obliterative phlebitis. Glucocorticoids form the basis of treatment.
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Número de páginas: 3
- Matilde Paulina Sinche Cueva
- Rosa Maria Rodrigues Pereira
- Giovanny Homero Jacome Verdugo
