Copyright ©The Author(s) 2018.
World J Nephrol. Jan 6, 2018; 7(1): 29-40
Published online Jan 6, 2018. doi: 10.5527/wjn.v7.i1.29
Table 1 Representative organ manifestations in IgG4-related disease
Organs adopted at the 1st International symposium in Boston in 2011
PancreasLymphoplasmacytic sclerosing pancreatitis
Eye/orbit/lacrimal glandsDacryadenitis/orbital inflammation/pseudotumour
Salivary glandsSialoadenitis/Mikulicz disease/Kuttner’s tumor
Mediastinum/retroperitoneumMediastinitis/retroperitoneal fibrosis/mesenteritis
KidneyTubulointerstitial nephritis/renal pyelitis
LungLung disease/inflammatory pseudotumor
Bile ducts/gall bladder/ liverSclerosing cholangitis/cholecystitis/hepatopathy
SkinSkin disease/pseudolymphoma
Limph nodeLymphadenopathy
Organs newly recognized after the Boston meeting
NerveInfraorbital nerve swelling
Paranasal sinusChronic rhinosinusitis
Testis/paratestisParatesticular pseudotumour
Urinary bladderInterstitial cystitis
Table 2 Conditions once regarded as individual disorders now recognized to be part of IgG4-related disease
Autoimmune pancreatitis (lymphoplasmacytic sclerosing pancreatitis)
Eosinophilic angiocentric fibrosis (affecting the orbits and upper respiratory tract)
Fibrosing mediastinitis
Hypertrophic pachymeningitis
Idiopathic hypocomplementemic tubulointerstitial nephritis with extensive tubulointerstitial deposits
Inflammatory pseudotumour (affecting the orbits, lungs, kidneys, and other organs)
Küttner’s tumor (affecting the submandibular glands)
Mikulicz’s disease (affecting the salivary and lacrimal glands)
Multifocal fibrosclerosis (commonly affecting the orbits, thyroid gland, retroperitoneum, mediastinum, and other tissues and organs)
Periaortitis and periarteritis
Inflammatory aortic aneurysm
Retroperitoneal fibrosis (Ormond’s disease)
Riedel’s thyroiditis
Sclerosing mesenteritis
Table 3 Mimickers of immunoglobulin G4-related disease
Antineutrophil cytoplasmic antibody-associated vasculitisAdenocarcinoma and squamous cell carcinomaCastleman’s disease
Granulomatosis with polyangiitisExtranodal marginal zone lymphomaCutaneous plasmocytosis
Eosinophilic granulomatosis with polyangiitisInflammatory myofibroblastic tumorErdheim-Chester disease
Microscopic polyangiitisLymphoplasmacytic lymphomaInflammatory bowel disease
SarcoidosisLymphoproliferative diseasePerforating collagenosis
Sjogren’s diseaseFollicular lymphomaPrimary sclerosing cholangitis
Rosai-Dorfman disease
Splenic sclerosing angiomatoid nodular transformation
Table 4 Clinical presentation of immunoglobulin G4-related disease per site of involvement
Organ systemNomenclatureClinical features
OrbitIgG4-related ophthalmic disease IgG4-related orbital inflammatory pseudo-tumor IgG4-related pan-orbital inflammation IgG4-related orbital myositisSwelling of orbital tissue and proptosis
Lacrimal glandIgG4-related dacryadenitisBilateral swelling of the glands and impaired production of secretion
Salivary glandIgG4-related sialoadenitis IgG4-related parotitis IgG4-related submandibular gland diseaseBilateral swelling of the glands and impaired production of secretion
ThyroidIgG4-related thyroid diseaseHypothyroidism, neck pain, dysphagia, dyspnea
LiverIgG4-related hepatopathyJaundice, right upper quadrant mass
Biliary tract and gall bladderIgG4-related sclerosing cholangitis IgG4-related cholecystitisJaundice, pruritus, cholestasis
Blood vesselsIgG4-related aortitis/periaortitis IgG4-related periarteritisChest pain, dyspnea
Retroperitoneal fibrosisIgG4-related retroperitoneal fibrosisFlank pain, obstructive symptoms, peripheral edema
KidneysIgG4-related kidney disease Tubulo-interstitial nephritis secondary to IgG4-related diseaseHematuria, proteinuria, hypocomplementemia, chronic renal failure
SkinIgG4-related skin diseasePapulonodular lesions, plaques, purpura
Table 5 Major histopathological features associated with immunoglobulin G4-related disease
Dense lymphoplasmacytic infiltrate
Fibrosis, arranged at least focally in a storiform pattern
Obliterative phlebitis
Phlebitis without obliteration of the lumen
Increased number of eosinophils
Table 6 Two proposed criteria for IgG4-TIN by the Mayo Clinic and the Japanese Society of Nephrology
CriterionThe Mayo Clinic criteriaJSN criteria
HistologyPlasma cell-rich TIN with > 10 IgG4+ plasma cells/HPF in the most concentrated field (mandatory criterion) TBM immune complex deposits by immunofluorescence, immunochemistry, and/or electron microscopyDense lymphoplasmacytic infiltrate with > 10 IgG4+ plasma cells/HPF and/or IgG4/IgG+ plasma cell ratio of > 40%; Characteristic storiform fibrosis
ImagingSmall peripheral low-attenuation cortical nodules, round or wedge-shaped lesions, or diffuse patchy involvementMultiple low-density lesions or enhanced CT, diffuse kidney enlargement, hypovascular solitary nodule, hypertrophic lesion of the renal pelvic wall
SerologyElevated serum IgG4 or total IgG levelElevated serum IgG4 or total IgG level
Clinical featuresNoneClinical or laboratory evidence of kidney damage
Other organ involvementCharacteristic findings of IgG4-RD in other organsCharacteristic findings of IgG4-RD in other organs
Definite IgG4-TINThe histologic feature and at least one other feature from imaging, serology or other organ involvementThe histologic feature (a and b) and at least two of other features from imaging, serology or other organ involvement