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IgG4-related disease

igg4 related disease, igg4 related disease icd 10
IgG4-related disease IgG4-RD, formerly known as IgG4-related systemic disease, is a chronic inflammatory condition characterized by tissue infiltration with lymphocytes and IgG4-secreting plasma cells, various degrees of fibrosis scarring and a usually prompt response to oral steroids In approximately 51–70% of people with this disease, serum IgG4 concentrations are elevated during an acute phase

It is a relapsing–remitting disease associated with a tendency to mass forming, tissue-destructive lesions in multiple sites, with a characteristic histopathological appearance in whichever site is involved Inflammation and the deposition of connective tissue in affected anatomical sites can lead to organ dysfunction, or even organ failure, if not treated

Early detection is important to avoid organ damage and potentially serious complications Treatment is recommended in all symptomatic cases of IgG4-RD and also in asymptomatic IgG4-RD involving certain anatomical sites


  • 1 Symptoms
    • 11 Individual organ manifestations
  • 2 Histology
    • 21 Submandibular gland research
  • 3 Diagnosis
  • 4 Treatment
    • 41 Induction of remission
    • 42 Maintenance
    • 43 Relapse
    • 44 Other interventions
    • 45 Trials
  • 5 Epidemiology
  • 6 Nomenclature
  • 7 See also
  • 8 References
  • 9 External links


IgG4-related disease has been described as an indolent condition Although possibly based on opinion rather than on objective assessments, symptoms, if any, are commonly described as mild in the medical literature This can be in spite of considerable underlying organ destruction People are often described as being generally well at the time of diagnosis, although some may give a history of weight loss

Pain is generally not a feature of the inflammation However it may occur as a secondary effect, for example due to either obstruction or compression

Often diagnosis is made due to the presence of painless swellings or mass lesions, or due to complications of masses, eg jaundice due to involvement of the pancreas, biliary tree or liver Symptoms are commonly attributed to other conditions and other diagnoses may have been made years before diagnosis, eg urinary symptoms in men attributed to common prostate conditions Lesions may also be detected incidentally on radiological images, but can be easily misdiagnosed as malignancies

Reported cases do include some significant symptoms or findings however:

SystemUncommon symptoms and complications
NeurologicalSeizures, paralysis or hemiparesis, cranial nerve palsies, sensorineural hearing loss, pituitary hormone deficiencies
EyeLoss of vision, proptosis
CardiovascularConstrictive pericarditis, heart block, ruptured aortic aneurysm, aortic dissection, carotid artery dissection, intracranial aneurysm, angina, sudden cardiac death
RespiratoryAirway obstruction, pleural effusion
GastrointestinalEsophageal obstruction, bowel obstruction
UrologicalRenal failure, hydronephrosis, testicular pain

Individual organ manifestations

IgG4-RD can involve one or multiple sites in the body With multiorgan involvement, the sites involved can be affected at the same time synchronously or at different unrelated periods metachronously

Several different diseases that have been known for many years are now considered to be manifestations of IgG4-RD These include:type 1 autoimmune pancreatitis, Riedel's thyroiditis, Mikulicz's disease, Küttner's tumor, inflammatory pseudotumors in various sites of the body, mediastinal fibrosis and some cases of retroperitoneal fibrosis

Nomenclature for individual organ involvement
Organ or sitePreferred namesPreviously used names
Head and neck
Salivary glandIgG4-related sialadenitis:IgG4-related submandibular gland disease,IgG4-related parotitisMikulicz's disease salivary and lacrimal glands, chronic sclerosing sialadenitis,
Küttner's tumour submandibular glands
OrbitIgG4-related ophthalmic disease IgG4-ROD including:IgG4-related dacryoadenitis lacrimal glands,IgG4-related orbital inflammation or IgG4-related orbital inflammatory pseudotumor,IgG4-related orbital myositis extraocular muscles,IgG4-related pan-orbital inflammation
Mikulicz's disease salivary and lacrimal glands,
Idiopathic orbital inflammatory disease, orbital pseudotumor
Paranasal sinuses Chronic sinusitis, Eosinophilic angiocentric fibrosis upper respiratory tract and orbit
PharynxIgG4-related pharyngitis 
Thyroid glandIgG4-related thyroid diseaseRiedel's thyroiditis, Riedel's struma
Soft tissues of the head and neck Idiopathic cervical fibrosis, sclerosing cervicitis, cervical fibrosclerosis
Central Nervous System
Pituitary glandIgG4-related hypophysitis:IgG4-related panhypophysitis all of pituitary gland,IgG4-related adenohypophysitis anterior pituitary,IgG4-related infundibuloneurohypophysitis posterior pituitary and pituitary stalkAutoimmune hypophysitis
MeningesIgG4-related pachymeningitis dura mater,
IgG4-related leptomeningitis arachnoid and pia mater
Idiopathic hypertrophic pachymeningitis
Chest and abdomen
PancreasIgG4-related pancreatitisType 1 autoimmune pancreatitis, lymphoplasmacytic sclerosing pancreatitis, 'chronic pancreatitis with diffuse irregular narrowing of the main pancreatic duct'
LungIgG4-related lung diseasePulmonary inflammatory pseudotumour
PleuraIgG4-related pleuritis 
LiverIgG4-related hepatopathy 
Bile ductIgG4-related sclerosing cholangitis 
GallbladderIgG4-related cholecystitis 
especially the infrarenal portion
IgG4-related aortitis,
IgG4-related periaortitis
Inflammatory aortic aneurysm,
Chronic sclerosing aortitis, chronic periaortitis
Branches of the aorta
including coronary, renal or iliac arteries
IgG4-related periarteritis 
PericardiumIgG4-related pericarditis 
MediastinumIgG4-related mediastinitisFibrosing mediastinitis, chronic sclerosing mediastinitis
RetroperitoneumIgG4-related retroperitoneal fibrosisRetroperitoneal fibrosis, Albarran-Ormond syndrome, Ormond's disease, perirenal fasciitis, Gerota's fasciitis/syndrome, periureteritis fibrosa, sclerosing lipogranuloma, sclerosing retroperitoneal granuloma, non-specific retroperitoneal inflammation, sclerosing retroperitonitis, retroperitoneal vasculitis with perivascular fibrosis
MesenteryIgG4-related mesenteritis subtypes are:mesenteric panniculitis, mesenteric lipodystrophy and retractile mesenteritisSclerosing mesenteritis, systemic nodular panniculitis, liposclerosis mesenteritis, mesenteric Weber–Christian disease, mesenteric lipogranuloma, xanthogranulomatous mesenteritis
BreastIgG4-related mastitisSclerosing mastitis
KidneyIgG4-related kidney disease IgG4-RKD:IgG4-related tubulointerstitial nephritis IgG4-TIN,membranous glomerulonephritis secondary to IgG4-related disease,IgG4-related renal pyelitis renal pelvisIdiopathic tubulointerstitial nephritis
ProstateIgG4-related prostatitis 
Vas deferensIgG4-related perivasal fibrosisChronic orchialgia
ScrotumIgG4-related paratesticular pseudotumor,
IgG4-related epididymo-orchitis
Paratesticular fibrous pseudotumor, inflammatory pseudotumor of the spermatic cord, pseudosarcomatous myofibroblastic proliferations of the spermatic cord, proliferative funiculitis, chronic proliferative periorchitis, fibromatous periorchitis, nodular periorchitis, reactive periorchitis, fibrous mesothelioma
Lymph nodesIgG4-related lymphadenopathy 
SkinIgG4-related skin diseaseAngiolymphoid hyperplasia with eosinophilia, cutaneous pseudolymphoma
NerveIgG4-related perineural disease 

This is not a complete list, as IgG4-RD can involve any site in the body

Other affected sites, confirmed on histology to be manifestations of IgG4-RD, include:heart; hard palate, esophagus, stomach, small intestine, rectum, adrenal gland, ovary, uterus, ureter, bladder, urachus, and synovium Approximately 1/3 of cases exhibit increases in blood eosinophil counts, either eosinophilia or hypereosinophilia

Radiologic evidence suggestive of involvement of the superior vena cava and seminal vesicle has been reported in confirmed cases of IgG4-RD


Whatever area of the body is involved, the hallmark histopathological features of IgG4-RD are:

  1. A dense lymphoplasmacytic lymphocytes and plasma cells infiltrate rich in IgG4-positive plasma cells
    • IgG4 immunostaining needs to be specifically requested and performed in order to detect IgG4-positive plasma cells
  2. Fibrosis, arranged at least focally in a storiform pattern
    • 'Storiform' is commonly referred to as meaning 'having a cartwheel pattern', but its literal meaning is the appearance of 'a woven matof rush or straw'
  3. Obliterative phlebitis
    • The venous channels are obliterated by a dense lymphoplasmacytic infiltrate, within both the venous walls and the lumen

Other histopathological features associated with IgG4-RD are:

  • Phlebitis without obliteration of the lumen
  • Tissue has increased numbers of eosinophils

Submandibular gland research

In an article from 1977, histological research into 349 cases of Küttner's tumor now known as 'IgG4-related sialadenitis' identified four distinct stages of the fibroinflammatory process:

  • Stage 1:Focal periductal around the salivary ducts infiltration of lymphocytes
  • Stage 2:Diffuse infiltration of lymphocytes and severe periductal fibrosis scarring around the salivary ducts
  • Stage 3:Prominent infiltration of lymphocytes, atrophy of parenchyma ie loss of functional areas due to shrinkage, and periductal sclerosis scarring resulting in hardening around the salivary ducts
  • Stage 4:Marked loss of and sclerosis hardening of the parenchyma functional area - similar to the process involved in cirrhosis where there is shrinkage and loss of functional areas of the liver

This may reflect the inflammatory process and development of fibrosis that occurs in other organs involved in IgG4-RD



The goal of treatment is the induction and maintenance of remission so as to prevent progression of fibrosis and organ destruction in affected organs

An international panel of experts have developed recommendations for the management of IgG4-RD They concluded that in all cases of symptomatic, active IgG4-RD that treatment is required Some cases with asymptomatic IgG4-RD also require treatment, as some organs tend to not cause symptoms until the late stages of disease Urgent treatment is advised with certain organ manifestations, such as aortitis, retroperitoneal fibrosis, proximal biliary strictures, tubulointerstitial nephritis, pachymeningitis, pancreatic enlargement and pericarditis

Induction of remission

In untreated patients with active disease, the recommended first-line agent for induction of remission is glucocorticoids unless contraindications exist Glucocorticoids characteristically result in a rapid and often dramatic improvement in clinical features and often a resolution of radiographic features However, where advanced fibrotic lesions have resulted in irreversible damage, the response to glucocorticoids and other current treatment options may be poor or even absent

Although not validated yet in clinical trials, the common induction regime is prednisolone 30–40 mg per day for 2–4 weeks, then gradually tapered over 3 to 6 months Recurrences during or after tapering of glucocorticoids are frequent however Steroid-sparing immunosuppressive agents might be considered, depending on local availability of these drugs, for use in combination with glucocorticoids from the start of treatment Steroid-sparing agents that have been used include rituximab, azathioprine, methotrexate, and cyclophosphamide, although trials are needed to ascertain the effectiveness of each drug in IgG4-RD


Following a successful induction of remission, maintenance therapy might be given in some cases, for example when there is a high risk of relapse or in patients with organ-threatening manifestations Common maintenancy therapy is prednisolone 25–5 mg per day, or use of a steroid-sparing agent instead


Relapses are common, and a previous history of relapse appears to be a strong predictor of future relapse When relapse occurs while off therapy and there has been a prolonged disease remission following initial glucocorticoid induction, then the relapse can usually be managed successfully with a re-induction strategy using glucocorticoids Introducing a steroid-sparing agent might also need to be considered for relapses; however, none has been tested in prospective, controlled studies, and evidence for their efficacy beyond that offered by concomitant glucocorticoid therapy is scarce

In one retrospective cohort study, baseline concentrations of serum IgG4, IgE and blood eosinophils were found to be independently predictive of relapse risk following treatment with rituximab with or without glucocorticoids; the higher the baseline values, the greater the relapse risk and the shorter the time to relapse

Other interventions

When organ involvement causes local mechanical problems, further organ-specific interventions may be necessary For example, when a tumefactive lesion cause obstruction of the bile duct, it may be necessary to insert a biliary stent to allow the bile to drain freely

Similarly, ureteral or vascular stents, surgical resection or radiotherapy may be considered for various different presenting problems


Research is also under way to evaluate the effect and safety of plasmablast-directed therapy with a monoclonal antibody XmAb5871 which inhibits B-cell function without depleting these immune cells XmAb5871 targets CD19 with its variable domain and has an Fc domain that has increased affinity to FcγRIIb


As recognition of IgG4-RD is relatively recent, there are limited studies on its epidemiology It is therefore difficult to make an accurate estimation of prevalence Furthermore, age of onset is almost impossible to estimate; age at diagnosis is frequently misused as the age of onset

A 2011 study estimated the incidence of IgG4-RD in Japan at 28–108/million population, with a median age of onset of 58 years


Names previously used for IgG4-RD:
IgG4-related systemic disease IgG4-RSD
IgG4-related sclerosing disease
IgG4-related systemic sclerosing disease
IgG4-related autoimmune disease
IgG4-associated multifocal systemic fibrosis
IgG4-associated disease
IgG4 syndrome
Hyper-IgG4 disease
Systemic IgG4-related plasmacytic syndrome
IgG4-positive multiorgan lymphoproliferative syndrome

Prior to 2011, IgG4-RD used to get mentioned in the medical literature under various different names

At the 2011 International Symposium on IgG4-Related Diseases, the consensus name of IgG4-related disease was endorsed for the condition This name had already been agreed upon as a consensus name among Japanese investigators, notably choosing not to use the term 'systemic' as that might lead to malignant tumours in other organs getting incorrectly diagnosed as being just another manifestation of the IgG4-related condition

However, some experts at the international symposium did express reservations about naming the disease after IgG4, as its role in pathogenesis is questionable and the use of serum IgG4 concentrations as a biomarker is unreliable

An expanded term, 'Immunoglobulin G4-related disease', has sometimes been used also However, this term was never referenced in the 2012 recommendations for nomenclature, and its use would appear to be erroneous

See also

  • IgG4-related ophthalmic disease
  • IgG4-related prostatitis
  • IgG4-related skin disease


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  41. ^ Terumi Kamisawa; Yuyang Tu; Naoto Egawa; Nobuhiro Sakaki; Jun-ichi Ishiwata; Kouji Tsuruta; Atsutake Okamoto; Toshio Takahashi; Morio Koike January 2001 "" Nippon Shokakibyo Gakkai Zasshi The Japanese Journal of Gastroenterology in Japanese The Japanese Society of Gastroenterology 98 1:15–24 doi:1011405/nisshoshi19649815 PMID 11201120 
  42. ^ a b Dai Inoue; Yoh Zen; Hitoshi Abo; Toshifumi Gabata; Hiroshi Demachi; Jyun Yoshikawa; Shiro Miyayama; Yasuni Nakanuma; Osamu Matsui November 2011 "Immunoglobulin G4–related Periaortitis and Periarteritis:CT Findings in 17 Patients" Radiology Radiological Society of North America 261 2:625–633 doi:101148/radiol11102250 PMID 21803920 
  43. ^ a b c Verghese George; Varaha Sairam Tammisetti; Venkateswar Rao Surabhi; Alampady K Shanbhogue July 2013 "Chronic Fibrosing Conditions in Abdominal Imaging" RadioGraphics Radiological Society of North America 33 4:1053–1080 doi:101148/rg334125081 PMID 23842972 
  44. ^ Jin Hee Kim; Jae Ho Byun; Seung Soo Lee; Hyoung Jung Kim; Moon-Gyu Lee January 2013 "Atypical Manifestations of IgG4-Related Sclerosing Disease in the Abdomen:Imaging Findings and Pathologic Correlations" American Journal of Roentgenology American Roentgen Ray Society 200 1:102–111 doi:102214/AJR128783 PMID 23255748 
  45. ^ Takashi Karashima; Yoshinori Taniguchi; Tsutomu Shimamoto; Tomoya Nao; Hiroshi Nishikawa; Satoshi Fukata; Masayuki Kamada; Keiji Inoue; Kentaro Oko; Hideki Nakajima; Shigetoshi Sano; Manabu Matsumoto; Naoto Kuroda; Yoshihiro Kamei; Taro Shuin 9 December 2014 "IgG4-related disease of the paratestis in a patient with Wells syndrome:a case report" Diagnostic Pathology 9:225 doi:101186/s13000-014-0225-5 PMC 4265405  PMID 25487870 
  46. ^ Hans Bösmüller; Claus Hann von Weyhern; Patrick Adam; Vedat Alibegovic; Gregor Mikuz; Falko Fend January 2011 "Paratesticular fibrous pseudotumor--an IgG4-related disorder" Virchows Archiv:the European Journal of Pathology European Society of Pathology 458 1:109–113 doi:101007/s00428-010-0995-4 PMID 20957491 
  47. ^ Yasuhito Hamaguchi; Manabu Fujimoto; Yukiyo Matsushita; Seiko Kitamura-Sawada; Mitsuhiro Kawano; Kazuhiko Takehara 2011 "IgG4-related skin disease, a mimic of angiolymphoid hyperplasia with eosinophilia" Dermatology Basel:Karger Publishers 223 4:301–305 doi:101159/000335372 PMID 22269779 
  48. ^ Yoshiki Tokura; Hiroaki Yagi; H Yanaguchi; Yuta Majima; Akira Kasuya; Taisuke Ito; M Maekawa; Hideo Hashizume November 2014 "IgG4-related skin disease" British Journal of Dermatology British Association of Dermatologists 171 5:959–967 doi:101111/bjd13296 PMID 25065694 
  49. ^ Dai Inoue; Yoh Zen; Yasuharu Sato; Hitoshi Abo; Hiroshi Demachi; Akio Uchiyama; Toshifumi Gabata; Osamu Matsui 2012 "IgG4-Related Perineural Disease" International Journal of Rheumatology 2012:401890 doi:101155/2012/401890 PMC 3317227  PMID 22523496 
  50. ^ Nicholas Andrew; Daniel Kearney; Nicole Sladden; Alastair Goss; Dinesh Selva April 2014 "Immunoglobulin G4-related disease of the hard palate" Journal of Oral and Maxillofacial Surgery American Association of Oral and Maxillofacial Surgeons 72 4:717–723 doi:101016/jjoms201308033 PMID 24268962 
  51. ^ Katie E Rollins; Samir P Mehta; Maria O'Donovan; Peter M Safranek 2011 "Gastric IgG4-Related Autoimmune Fibrosclerosing Pseudotumour:A Novel Location" ISRN Gastroenterology 2011 2011:873087 doi:105402/2011/873087 PMC 3168566  PMID 21991533 
  52. ^ Younghwan Ko; Ji Young Woo; Jeong Won Kim; Hye Sook Hong; Ik Yang; Yul Lee; Daehyun Hwang; Seon Jeong Min Sep–Oct 2013 "An Immunoglobulin G4-Related Sclerosing Disease of the Small Bowel:CT and Small Bowel Series Findings" Korean Journal of Radiology 14 5:776–780 doi:103348/kjr2013145776 PMC 3772257  PMID 24043971 
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  64. ^ "Xencor Initiates Two Phase 2 Trials of XmAb5871 in IgG4-Related Disease and Systemic Lupus Erythematosus" PR Newswire 7 March 2016 Retrieved 23 July 2016 The primary objective of the study is to evaluate the effect of every other week IV administration of XmAb5871 on the IgG4-RD Responder Index RI in patients with active IgG4-RD 
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External links

ClassificationV · T · D
  • ICD-10:M35
External resources
  • Orphanet:284264

  • Overview of IgG4-related disease - UpToDate's article on IgG4-related disease
  • DermNet NZ entry

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IgG4-related disease

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