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IgG4-related diseases

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 International Symposium on IgG4-related disease (IS IgG4-RD 2015; 2012).
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Guidelines

1.Screening and diagnosis

Diagnostic criteria: view a previously unrecognized organ or site as involved by IgG4-related disease if the following criteria areMET :
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
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2.Classification and risk stratification

Nomenclature: 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

3.Diagnostic investigations

Evaluation: elicit a full clinical history, perform physical examination and obtain selected laboratory and appropriate imaging studies for accurate assessment of IgG4-RD.
B

4.Diagnostic procedures

Biopsy: perform biopsy to confirm the diagnosis of IgG4-related disease and exclude malignancies and other IgG4-RD mimics.
B

5.Medical management

Indications for treatment
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

6.Follow-up and surveillance

Management of relapse: offer re-treatment with corticosteroids in patients with a relapse following successful remission induction. Consider offering a steroid-sparing agent following relapse for continuation in the remission maintenance period.
B