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<p>IgG4-related disease (IgG4-RD) is a&#xa0;systemic fibroinflammatory condition characterized by multiorgan involvement and a&#xa0;highly heterogeneous clinical presentation that frequently mimics autoimmune, inflammatory, and malignant disorders. This review provides a&#xa0;clinically oriented diagnostic framework guiding physicians from initial symptoms to confirmed diagnosis while highlighting major differential diagnostic pitfalls. After defining the disease entity, typical organ manifestations and clinical phenotypes are outlined, followed by an overview of the current American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) classification criteria and the diagnostic relevance of histopathology. Particular emphasis is placed on the role of fluorodeoxyglucose position-emission tomography–computed tomography (FDG PET-CT) as the most comprehensive imaging modality for detecting subclinical organ involvement and guiding biopsy site selection. The diagnostic value and limitations of serum IgG4 concentrations are critically discussed, as elevated levels lack sufficient sensitivity and specificity and cannot establish the diagnosis in isolation. A&#xa0;major focus is the clinical overlap with important differential diagnoses such as eosinophilic granulomatosis with polyangiitis (Churg–Strauss syndrome), Sjögren’s syndrome, giant cell arteritis, and Erdheim–Chester disease. The article also highlights vascular involvement across different vessel sizes, supporting the concept of a&#xa0;variable-vessel vasculitis phenotype. Glucocorticoids remain first-line therapy, and rapid clinical response strongly supports the diagnosis, whereas steroid resistance should prompt reconsideration of alternative conditions. Overall, accurate diagnosis requires integration of clinical, radiologic, and histologic findings within a&#xa0;multidisciplinary framework to avoid misdiagnosis and inappropriate treatment.</p>

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Vom Symptom zur Diagnose

  • 期刊论文

  • 发表日期：

    2026年8月4日

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  • Eva Rath,
  • Eva Manger

摘要

IgG4-related disease (IgG4-RD) is a systemic fibroinflammatory condition characterized by multiorgan involvement and a highly heterogeneous clinical presentation that frequently mimics autoimmune, inflammatory, and malignant disorders. This review provides a clinically oriented diagnostic framework guiding physicians from initial symptoms to confirmed diagnosis while highlighting major differential diagnostic pitfalls. After defining the disease entity, typical organ manifestations and clinical phenotypes are outlined, followed by an overview of the current American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) classification criteria and the diagnostic relevance of histopathology. Particular emphasis is placed on the role of fluorodeoxyglucose position-emission tomography–computed tomography (FDG PET-CT) as the most comprehensive imaging modality for detecting subclinical organ involvement and guiding biopsy site selection. The diagnostic value and limitations of serum IgG4 concentrations are critically discussed, as elevated levels lack sufficient sensitivity and specificity and cannot establish the diagnosis in isolation. A major focus is the clinical overlap with important differential diagnoses such as eosinophilic granulomatosis with polyangiitis (Churg–Strauss syndrome), Sjögren’s syndrome, giant cell arteritis, and Erdheim–Chester disease. The article also highlights vascular involvement across different vessel sizes, supporting the concept of a variable-vessel vasculitis phenotype. Glucocorticoids remain first-line therapy, and rapid clinical response strongly supports the diagnosis, whereas steroid resistance should prompt reconsideration of alternative conditions. Overall, accurate diagnosis requires integration of clinical, radiologic, and histologic findings within a multidisciplinary framework to avoid misdiagnosis and inappropriate treatment.