Table of contents
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).
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
2.Classification and risk stratification
Nomenclature: use the following nomenclature for IgG4-RD:
Type 1 autoimmune pancreatitis (IgG4-related pancreatitis)
IgG4-related ophthalmic disease (the general term for the periocular manifestations of this disease)
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
Thyroid (Riedel's thyroiditis)
IgG4-related thyroid disease
IgG4-related retroperitoneal fibrosis
IgG4-related skin disease
IgG4-related hepatopathy (refers to liver involvement that is distinct from biliary tract involvement)
IgG4-related lung disease
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)
Evaluation: elicit a full clinical history, perform physical examination and obtain selected laboratory and appropriate imaging studies for accurate assessment of IgG4-RD.
Biopsy: perform biopsy to confirm the diagnosis of IgG4-related disease and exclude malignancies and other IgG4-RD mimics.
Indications for treatment
Initiate treatment in all patients (some urgently) with symptomatic active IgG4-RD.
Initiate treatment in certain patients with asymptomatic IgG4-RD.
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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.