Show pageBacklinksCite current pageExport to PDFBack to top This page is read only. You can view the source, but not change it. Ask your administrator if you think this is wrong. ====== Immunoglobulin G4-related disease (IgG4-RD) ====== [[Immunoglobulin G4]]-related disease (IgG4-RD) is a chronic, systemic, fibroinflammatory disorder that can affect virtually any organ. It is characterized histologically by a dense lymphoplasmacytic infiltrate, storiform fibrosis, and obliterative phlebitis. ===== Histopathological Features ===== * Dense lymphoplasmacytic infiltrate * Storiform fibrosis * Obliterative phlebitis * IgG4+ plasma cells >10/high-power field (HPF) * IgG4+/IgG+ cell ratio >40% ===== Frequently Involved Organs ===== * Pancreas → Autoimmune pancreatitis (type 1) * Biliary tract → IgG4-related sclerosing cholangitis * Salivary and lacrimal glands → Mikulicz’s disease * Retroperitoneum → Retroperitoneal fibrosis * Kidneys → Tubulointerstitial nephritis * Lungs → Inflammatory pseudotumor * Orbit → IgG4-related ophthalmic disease * CNS → Hypertrophic pachymeningitis ===== Clinical Features ===== * Painless swelling or mass-like lesions * Organ dysfunction depending on site * Constitutional symptoms (rare): weight loss, low-grade fever ===== Diagnosis ===== * Clinical suspicion based on organ involvement * Imaging showing mass or enlargement * Histopathology confirming IgG4-RD criteria * Immunohistochemistry: IgG4+ plasma cells and IgG4+/IgG+ ratio * Serum IgG4 levels (elevated in ~60–70%) ===== Treatment ===== * First-line: Glucocorticoids (e.g., prednisolone) * Steroid-sparing agents: azathioprine, mycophenolate mofetil * Refractory cases: Rituximab ===== Differential Diagnosis ===== * Lymphoma * Sarcoidosis * Granulomatosis with polyangiitis (Wegener) * Infectious or neoplastic diseases ===== Case reports ===== Zeng et al. present a case report of a man in his 30s with IgG4-related ophthalmic disease (IgG4-ROD) and an orbital hemangioma, claiming extreme rarity of this co-occurrence ((Zeng H, Peng X, He W. Immunoglobulin G4-related ophthalmic disease with orbital deep hemangioma: A case report. J Int Med Res. 2025 Jun;53(6):3000605251345239. doi: 10.1177/03000605251345239. Epub 2025 Jun 10. PMID: 40494658.)). While interesting, the manuscript never explains why this coexistence matters pathophysiologically or therapeutically—other than as a curiosity. Without a rationale for clinical relevance, the reader is left wondering: so what? 🧪 2. Diagnostic Flimsiness The diagnosis of IgG4-related disease—a notoriously tricky entity requiring a combination of histopathological, serological, and imaging criteria—is asserted without rigorous justification: No detailed histopathological scoring (e.g., IgG4+/IgG+ plasma cell ratio). No mention of serum IgG4 level thresholds or systemic involvement. Vague description of fibrosis or phlebitis. → In essence: They label it IgG4-ROD without ticking the diagnostic boxes. 🩻 3. Orbital Hemangioma = Red Herring The coexistence with an orbital hemangioma is presented as if it's a new syndrome. But: Orbital hemangiomas are common vascular lesions. There is no mechanistic link proposed between the two conditions. No compelling imaging or immunohistochemistry tying both pathologies together. It reads like a case of a guy with two things in the same place, not a true overlap syndrome. 🛠️ 4. Therapeutic Vagueness They describe treatment with glucocorticoids (as per IgG4 disease protocols), but: No response curve. No discussion of tapering, resistance, or follow-up. No exploration of whether the hemangioma itself required any intervention. It ends up looking like a missed opportunity to inform on management nuances of these lesions in tandem. 📉 5. Formatting & Style Deficiencies The writing is uncritical and descriptive, lacking reflection. References are outdated or scant. Figures (if any) are not described in a way that adds value. 🔚 Bottom Line This is a classic case report written for the sake of publishing a rare association—without analysis, insight, or educational depth. Its contribution to clinical practice is minimal, and its diagnostic reasoning is more decorative than robust. Final diagnosis: Case report syndrome—benign but self-limiting immunoglobulin_g4-related_disease.txt Last modified: 2025/06/11 07:10by administrador