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[IgG4-related disease: a case report].
Luis Francisco Pineda-Galindo1, Olga Lidia Vera-Lastra, Lucía Carrazco-Ibarra
1Hospital de Especialidades "Dr. Antonio Fraga Mouret", Centro Médico Nacional La Raza, Instituto Mexicano del Seguro Social, Ciudad de México, México uigi_doc@yahoo.com.
Revista Medica Del Instituto Mexicano Del Seguro Social
|December 1, 2017
Summary
Immunoglobulin G4-related disease (IgG4-RD) is an inflammatory condition. A patient with Mikulicz's disease demonstrated the characteristic histological findings required for an IgG4-RD diagnosis.
Area of Science:
- Immunology
- Pathology
- Rheumatology
Background:
- Immunoglobulin G4-related disease (IgG4-RD) is a multisystemic fibroinflammatory condition.
- It is characterized by elevated serum IgG4 levels and IgG4-positive plasma cell infiltration in affected tissues.
- Commonly affected organs include the salivary glands, lacrimal glands, pancreas, lymph nodes, lungs, and kidneys.
Observation:
- Mikulicz's disease is a chronic inflammatory condition affecting the lacrimal and salivary glands.
- This case study presents a patient diagnosed with Mikulicz's disease.
- The patient's condition exhibited specific histological features relevant to IgG4-RD diagnosis.
Findings:
- Diagnosis of IgG4-RD relies on characteristic histological findings.
- These include dense lymphocyte and plasmacyte infiltration, focal fibrosis, and phlebitis.
- Key diagnostic criteria involve >10 IgG4+ cells/high power field and/or an IgG4/IgG ratio >40%.
Implications:
- This case highlights the overlap between Mikulicz's disease and IgG4-RD.
- Accurate diagnosis is crucial for appropriate treatment and management of IgG4-RD.
- Recognizing these histological patterns aids in differentiating IgG4-RD from other conditions.
