Table of contents
Expand All Topics
IgG4-related diseases
Guidelines
Key sources
The following summarized guidelines for the evaluation and management of IgG4-related diseases are prepared by our editorial team based on guidelines from the American Academy of Family Physicians (AAFP 2024) and the International Symposium on IgG4-related disease (IS IgG4-RD 2015,2012).
1
2
3
4
Screening and diagnosis
Diagnostic criteria: as per IS IgG4-RD 2012 guidelines, view a previously unrecognized organ or site as involved by IgG4-related disease if the following criteria are met:
characteristic histopathological findings with an elevated IgG4-positive plasma cells and IgG4-to-IgG ratio
elevated serum IgG4 levels
effective response to corticosteroid therapy
other organ involvement consistent with IgG4-related disease.
E
Classification and risk stratification
Nomenclature: as per IS IgG4-RD 2012 guidelines, use the following nomenclature for IgG4-RD:
Situation
Guidance
Pancreas
Type 1 autoimmune pancreatitis (IgG4-related pancreatitis)
Eye
IgG4-related ophthalmic disease (the general term for the periocular manifestations of this disease)
Lacrimal glands
IgG4-related dacryoadenitis
Orbital soft tissue (orbital inflammatory pseudotumor)
IgG4-related orbital inflammation (or IgG4-related orbital inflammatory pseudotumor)
Extraocular muscle disease
IgG4-related orbital myositis
Orbit with involvement of multiple anatomic structures
IgG4-related pan-orbital inflammation (includes lacrimal gland disease, extraocular muscle involvement, and other potential intraorbital complications)
Salivary glands (parotid and submandibular glands)
IgG4-related sialadenitis or, more specifically, IgG4-related parotitis or IgG4-related submandibular gland disease
Pachymeninges
IgG4-related pachymeningitis
Hypophysis
IgG4-related hypophysitis
Thyroid (Riedel's thyroiditis)
IgG4-related thyroid disease
Aorta
IgG4-related aortitis/periaortitis
Arteries
IgG4-related periarteritis
Mediastinum
IgG4-related mediastinitis
Retroperitoneum
IgG4-related retroperitoneal fibrosis
Mesentery
IgG4-related mesenteritis
Skin
IgG4-related skin disease
Lymph node
IgG4-related lymphadenopathy
Bile ducts
IgG4-SC
Gallbladder
IgG4-related cholecystitis
Liver
IgG4-related hepatopathy (refers to liver involvement that is distinct from biliary tract involvement)
Lung
IgG4-related lung disease
Pleura
IgG4-related pleuritis
Pericardium
IgG4-related pericarditis
Kidney
IgG4-related kidney disease (tubulointerstitial nephritis secondary to IgG4-related disease and membranous glomerulonephritis secondary to IgG4-related disease for specific renal complications, IgG4-related renal pyelitis for the involvement of the renal pelvis)
Breast
IgG4-related mastitis
Prostate
IgG4-related prostatitis
E
Diagnostic investigations
General principles: as per IS IgG4-RD 2015 guidelines, elicit a full clinical history, perform physical examination and obtain selected laboratory and appropriate imaging studies for accurate assessment of IgG4-RD.
B
More topics in this section
Diagnostic imaging
Diagnostic procedures
Sialendoscopy: as per AAFP 2024 guidelines, perform sialendoscopy for pain relief and diagnosis in patients with chronic obstructive sialadenitis.
B
More topics in this section
Biopsy
Medical management
Indications for treatment
As per IS IgG4-RD 2015 guidelines:
Initiate treatment in all patients (some urgently) with symptomatic active IgG4-RD.
B
Initiate treatment in certain patients with asymptomatic IgG4-RD.
B
More topics in this section
Induction therapy
Maintenance therapy
Sialagogues