What are the guideline sources, guidelines and references for IgG4-related diseases ?
Guideline 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). 1 2 3 Guidelines 1. Screening and diagnosis Diagnostic criteria: 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 2. Classification and risk stratification https://web.pathway.md/diseases/rec1IJPTTQ2AyaNLn 1/4 6/24/23, 2:28 PM IgG4-related diseases Pathway Nomenclature: Use the following nomenclature for IgG4-related diseases: Situation Guidance Type 1 autoimmune pancreatitis (IgG4- related pancreatitis) Pancreas IgG4-related ophthalmic disease (the general term for the periocular manifestations of this disease) Eye IgG4-related dacryoadenitis Lacrimal glands IgG4-related orbital inflammation (or IgG4- related orbital inflammatory pseudotumor) Orbital soft tissue (orbital inflammatory pseudotumor) IgG4-related orbital myositis Extraocular muscle disease IgG4-related pan-orbital inflammation (includes lacrimal gland disease, extraocular muscle involvement, and other potential intraorbital complications) Orbit with involvement of multiple anatomic structures IgG4-related sialadenitis or, more specifically, IgG4-related parotitis or IgG4-related submandibular gland disease Salivary glands (parotid and submandibular glands) IgG4-related pachymeningitis Pachymeninges IgG4-related hypophysitis Hypophysis IgG4-related thyroid disease Thyroid (Riedel's thyroiditis) IgG4-related aortitis/periaortitis Aorta IgG4-related periarteritis Arteries IgG4-related mediastinitis Mediastinum IgG4-related retroperitoneal fibrosis Retroperitoneum IgG4-related mesenteritis Mesentery IgG4-related skin disease Skin IgG4-related lymphadenopathy Lymph node IgG4-related sclerosing cholangitis Bile ducts IgG4-related cholecystitis Gallbladder IgG4-related hepatopathy (refers to liver involvement that is distinct from biliary tract Liver https://web.pathway.md/diseases/rec1IJPTTQ2AyaNLn 2/4 6/24/23, 2:28 PM IgG4-related diseases Pathway involvement) IgG4-related lung disease Lung IgG4-related pleuritis Pleura IgG4-related pericarditis Pericardium 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) Kidney IgG4-related mastitis Breast IgG4-related prostatitis. E Prostate 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-related diseases. B 4. Diagnostic procedures Biopsy: perform biopsy to confirm the diagnosis of IgG4-related disease and exclude malignancies and other IgG4-related diseases mimics. B 5. Medical management Indications for treatment: Initiate treatment in all patients (some urgently) with symptomatic active IgG4-related diseases. B Initiate treatment in certain patients with asymptomatic IgG4-related diseases. B Induction therapy: Administer corticosteroids as first-line therapy for remission induction in all patients with active, untreated IgG4-related diseases, unless contraindicated. B Offer a combination of corticosteroids and a steroid-sparing immunosuppressive agent from the start of treatment in certain patients with IgG4-related diseases, because corticosteroid monotherapy will ultimately fail to control the disease and long-term corticosteroid toxicities pose a high risk to patients. B https://web.pathway.md/diseases/rec1IJPTTQ2AyaNLn 3/4 6/24/23, 2:28 PM IgG4-related diseases Pathway Maintenance therapy: consider offering maintenance therapy in certain patients with IgG4- related diseases following a successful course of induction therapy. C 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 References 1. A Khosroshahi, Z S Wallace, J L Crowe et al. International Consensus Guidance Statement on the Management and Treatment of IgG4 Related Disease. Arthritis Rheumatol. 2015 Jul;67 7 1688 99. Open 2. Vikram Deshpande, Yoh Zen, John Kc Chan et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol. 2012 Sep;25 9 1181 92. Open 3. John H Stone, Arezou Khosroshahi, Vikram Deshpande et al. Recommendations for the nomenclature of IgG4-related disease and its individual organ system manifestations. Arthritis Rheum. 2012 Oct;64 10 3061 7. Open https://web.pathway.md/diseases/rec1IJPTTQ2AyaNLn 4/4