Table 1 The clinical spectrum of IgG4-related disease
From: IgG4-related disease—rare but you should not forget it
Organ involvement | Manifestations | Clinical presentation/radiological or laboratory findings |
|---|---|---|
Head and neck | ||
Eye and orbit | Dacryoadenitis Dacryocystitis Orbital myositis Orbital pseudotumor Scleritis (rare) Uveitis (rare) | Lacrimal gland enlargement (typically bilateral) Syndrome sicca Diplopia Proptosis Ptosis Red eye, ocular pain |
Salivary glands | Sialadenitis of submandibular, parotid and/or sublingual | Glandular swelling Typically, bilateral Nontender or occasionally mild tender Syndrome sicca |
Ear, nose, and throat | Chronic rhinosinusitis Midline destructive lesion Pharyngitis Supraglottic stenosis Vocal cord lesion | Allergic rhinitis symptoms, sinusitis, nasal polyps, rhinorrhea, nasal obstruction Nasal septum perforation (rare) Anosmia, middle ear effusion Hoarseness, Dyspnea |
Thyroid gland | IgG4-related thyroiditis (Riedel) | Neck mass (woody) and pressure Hypothyroidism Recurrent laryngeal nerve palsy |
Thorax | ||
Lungs and pleura | Parenchymal lung disease Pleural disease | Thickening of the bronco vascular bundles Interstitial lung disease, ground glass opacities Pulmonary nodules Pleural: effusion, thickening or nodules |
Heart and pericardium | Coronary arteritis Pericarditis Pseudotumor | Perivasculitis around coronary arteries Coronary artery aneurysms Pericardial effusion Pericardial thickening Cardiac mass |
Mediastinum | Fibrosing mediastinitis Paravertebral mass | Dyspnea, chest pain Mediastinal mass with compression of structures Paravertebral soft tissue, usually right-sided and located between T8-T11 |
Great vessels | Aortitis Periaortitis | Aortic wall thickening and enhancement Perivascular soft tissue around great vessels |
Breast | IgG4-related mastitis | Painless breast mass |
Abdomen and pelvis | ||
Pancreas | Autoimmune pancreatitis type 1 Pseudotumor | Obstructive jaundice, abdominal pain, diabetes mellitus, malabsorption Diffuse pancreatic enlargement (sausage-shaped pancreas) Capsule-like pancreatic rim Pancreatic atrophy (long-standing disease) Irregular narrowing of pancreatic duct Pancreatic mass (simulating adenocarcinoma) |
Hepatobiliary system | Sclerosing cholangitis Sclerosing cholecystitis Hepatic inflammatory pseudotumor | Jaundice, weight loss, abdominal pain |
Mass mimicking cholangiocarcinoma | ||
Intra- and extrahepatic biliary ductal dilatation | ||
Gallbladder wall thickening | ||
Hepatic mass, transaminitis | ||
Kidneys | Tubulointerstitial nephritis Membranous glomerulonephritis | Elevated creatinine, proteinuria, hematuria, nephritic/nephrotic syndrome Diffuse kidney enlargement Renal cortical hypodensities Renal pelvic wall thickening Renal atrophy (long-standing disease) |
Retroperitoneum | Retroperitoneal fibrosis (Ormond’s disease) Aortitis and periaortitis | Back/flank pain, leg edema, hydronephrosis, deep venous thrombosis, varicocele Retroperitoneal mass or fat stranding Periaortic inflammation involving all or parts of the aorta distal to the renal arteries, extending to the iliac arteries |
Nervous system | ||
Pituitary | Hypophysitis | Hypopituitarism, diabetes insipidus, headache Pituitary enlargement Thickened pituitary stalk |
Meninges | Hypertrophic pachymeningitis | Headache, cranial nerve palsies, vision disturbance, sensorineural hearing loss, seizures, motor weakness, limb numbness Hypertrophic and nodular meningeal thickening Hydrocephalus |
Central nervous system | Dementia, hemiparesis, multifocal neurological defects | |
Peripheral nerves | Often asymptomatic—incidental radiological finding | Perineuritis, most typically of the infraorbital nerve |
Lymph nodes | Often asymptomatic | Lymph node enlargement (generalized or localized), generally 1–3 cm in diameter, non-tender Does not have a preference for any set of lymph node |