Table 2.
IgG4-related disease (IgG4-RD): summary of clinical manifestations by organ system.
| Organ | Clinical and Radiological Findings | Differential Diagnosis |
|---|---|---|
| Orbital and peri-orbital area | Orbital pseudotumor; dacryoadenitis; dacrocystitis; Orbital mass lesions | Lymphoma; Graves’disease; Granulomatosis with polyangiitis (Wegener’s); Sarcoidosis |
| Meninges | Hypertrophic pachimeningitis | Inflammatory myofibroblastic tumor; Granulomatosis with polyangiitis (Wegener’s); Giant cell arteritis; Langerhans cell histiocytosis; Sarcoidosis |
| Pituitary gland | Hypophysitis | Neoplasms; Histiocytosis; Hypophysitis: Primary or Secondary (sarcoidosis, ipilimumb-induced) |
| Salivary glands | Sialoadenitis (Mikulicz Disease) | Lymphoma; Sjögren’s syndrome; Sarcoidosis; Sialodocholithiasis |
| Upper airways | Nasal polyps, allergic rhinitis, nasal obstruction, rhinorrhea, anosmia, chronic sinusitis, eosinophiic angiocentric fibrosis | Allergic disease; Churg-Strauss syndrome; Granulomatosis with polyangiitis (Wegener’s); Sarcoma |
| Thyroid gland | Hypothyroidism; thyroid gland enlargement; Riedel’s thyroiditis | Thyroid lymphoma; Differentiated thyroid carcinoma; Other malignancy |
| Cardiovascular system | Constrictive pericarditis; peri-aortitis; inflammatory aneurysm; coronary arteritis | |
| Respiratory system | Parenchymal lung consolidations, inflammatory pseudotumor, central airway disease, interstitial lung disease, pleural effusion, pleural thickening, pleural nodules or masses | Malignancy; Inflammatory myofibroblastic tumor; Sarcoidosis; Castleman’s disease; Lymphomatoid disease; Pleural malignancy; Non specific pleuritis; Interstitial lung disease |
| Mediastinum and Retroperitoneum | Lymphoadenopathy; retroperitoneum fibrosis | Sarcoidosis; Castleman’s disease; Lymphomatoid disease |
| Gastrointestinal system | Autoimmune pancreatitis; Inflammatory mesenteritis; Sclerosing cholangitis; Mass lesions liver | Pancreatic cancer; Cholangiocarcinoma; Primary sclerosing cholangitis; Hepatocarcinoma |
| Haematopoietic-Lymphatic system | Lymphoadenopaty; Eosinofilia; Polyclonal hypergammaglobulinemia | Myeloma; Lymphomatoid and Myeloid disease |
| Urinary system | Tubulointerstitial nephritis; Tumoral lesions | Lymphoma; Renal cell carcinoma; Paucimmune Necrotizing Glomerulonephritis; Sarcoidosis Sjögren’s syndrome Systemic lupus erythematosus |