IgG4-related disease

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IgG4-related disease
Other namesIgG4-related systemic disease
Low power view of IgG4-related prostatitis.jpg
Low power view of IgG4-related prostatitis. The prostatic stroma shows a dense inflammatory infiltrate and fibrosis (H&E, 100x)
Specialty Immunology/Rheumatology

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. [1] [2] [3]

Contents

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, organ failure, or even death if not treated. [4]

Early detection is important to avoid organ damage and potentially serious complications. [5] Treatment is recommended in all symptomatic cases of IgG4-RD and also in asymptomatic IgG4-RD involving certain anatomical sites. [4] [6]

Signs and symptoms

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. [7] 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 inflammation. However, it may occur as a secondary effect, for example, due to either obstruction or compression.

Laboratory levels of IgG4 greater than 135 mg/dL

Moreover, diagnosis is made due to the presence of painless swellings or mass lesions, or due to complications of masses, e.g. 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, e.g. 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.[ citation needed ]

Reported cases do include some significant symptoms or findings however:

SystemUncommon symptoms and complications
Neurological Seizures, [8] [9] paralysis or hemiparesis, [8] cranial nerve palsies, [8] sensorineural hearing loss, [10] pituitary hormone deficiencies [11]
EyeLoss of vision, [8] proptosis [12]
Cardiovascular Constrictive pericarditis, [13] heart block, [14] ruptured aortic aneurysm, [15] [16] aortic dissection, [17] carotid artery dissection, [18] intracranial aneurysm, [19] angina, [20] sudden cardiac death [21] [22]
Respiratory Airway obstruction, [23] pleural effusion [24] [25]
GastrointestinalEsophageal obstruction, [26] [27] bowel obstruction [28]
Urological Renal failure, [29] hydronephrosis, [29] [30] testicular pain [31]

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, interstitial nephritis, 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. [32] [33]

Nomenclature for individual organ involvement [2] [34]
Organ or sitePreferred namesPreviously used names
Head and neck
Salivary gland IgG4-related sialadenitis:
IgG4-related submandibular gland disease,
IgG4-related parotitis
 Mikulicz's disease (salivary and lacrimal glands), [35] chronic sclerosing sialadenitis,
Küttner's tumour (submandibular glands)
Orbit IgG4-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), [35]
Idiopathic orbital inflammatory disease, orbital pseudotumor
Paranasal sinuses [36]  Chronic sinusitis, Eosinophilic angiocentric fibrosis (upper respiratory tract and orbit) [37]
Pharynx IgG4-related pharyngitis [38]  
Thyroid gland IgG4-related thyroid disease Riedel's thyroiditis, Riedel's struma
Soft tissues of the head and neck Idiopathic cervical fibrosis, [39] sclerosing cervicitis, cervical fibrosclerosis
Central Nervous System
Pituitary gland IgG4-related hypophysitis:
IgG4-related panhypophysitis (all of pituitary gland),
IgG4-related adenohypophysitis (anterior pituitary),
IgG4-related infundibuloneurohypophysitis (posterior pituitary and pituitary stalk)
 Autoimmune hypophysitis
Meninges IgG4-related pachymeningitis (dura mater),
IgG4-related leptomeningitis [40] [41] (arachnoid and pia mater)		 Idiopathic hypertrophic pachymeningitis
Chest and abdomen
Pancreas IgG4-related pancreatitisType 1 autoimmune pancreatitis, lymphoplasmacytic sclerosing pancreatitis, 'chronic pancreatitis with diffuse irregular narrowing of the main pancreatic duct' [42]
Lung IgG4-related lung diseasePulmonary inflammatory pseudotumour
Pleura IgG4-related pleuritis 
Liver IgG4-related hepatopathy 
Bile duct IgG4-related sclerosing cholangitis 
Gallbladder IgG4-related cholecystitis 
Aorta
(especially the infrarenal portion)		IgG4-related aortitis,
IgG4-related periaortitis [43] 		Inflammatory aortic aneurysm,
Chronic sclerosing aortitis, chronic periaortitis.
Branches of the aorta
(including coronary, [36] renal or iliac arteries)		IgG4-related periarteritis [43]  
Pericardium IgG4-related pericarditis 
Mediastinum IgG4-related mediastinitisFibrosing mediastinitis, chronic sclerosing mediastinitis
Retroperitoneum IgG4-related retroperitoneal fibrosis Retroperitoneal 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. [44]
Mesentery IgG4-related mesenteritis (subtypes are: mesenteric panniculitis, mesenteric lipodystrophy and retractile mesenteritis) [44] Sclerosing mesenteritis, systemic nodular panniculitis, liposclerosis mesenteritis, mesenteric Weber–Christian disease, mesenteric lipogranuloma, xanthogranulomatous mesenteritis. [44]
Breast IgG4-related mastitisSclerosing mastitis
Genitourinary
Kidney IgG4-related kidney disease (IgG4-RKD):
IgG4-related tubulointerstitial nephritis (IgG4-TIN),
membranous glomerulonephritis secondary to IgG4-related disease,
IgG4 plasma cell arteritis
IgG4-related renal pyelitis (renal pelvis)
Idiopathic hypocomplementemic tubulointerstitial nephritis
Prostate IgG4-related prostatitis  
Vas deferens IgG4-related perivasal fibrosis [31] [45] Chronic orchialgia
Scrotum IgG4-related paratesticular pseudotumor, [34] [46]
IgG4-related epididymo-orchitis [34] 		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 [47]
Other
Lymph nodes IgG4-related lymphadenopathy 
Skin IgG4-related skin disease Angiolymphoid hyperplasia with eosinophilia, [48] cutaneous pseudolymphoma [49]
Nerve IgG4-related perineural disease [34] [50]  

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; [14] hard palate, [51] esophagus, [26] [27] stomach, [52] small intestine, [53] rectum, [54] adrenal gland, [55] ovary, [56] uterus, [24] ureter, [57] bladder, [58] urachus, [59] and synovium. [60] Approximately 1/3 of cases exhibit increases in blood eosinophil counts, either eosinophilia or hypereosinophilia.[ citation needed ]

Radiologic evidence suggestive of involvement of the superior vena cava [14] and seminal vesicle [61] has been reported in confirmed cases of IgG4-RD.

Histology

Whatever area of the body is involved, the hallmark histopathological features of IgG4-RD are: [3] [32] [33]

  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 [3] 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 mat [Latin: storea] (of rush or straw)'.
  3. Obliterative phlebitis.
    • The venous channels are obliterated by a dense lymphoplasmacytic infiltrate, within both the venous walls and the lumen. [3]

Other histopathological features associated with IgG4-RD are:

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: [62]

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

Diagnosis

Diagnosis requires tissue biopsy of an affected organ with characteristic histological findings, a comprehensive medical history and physical examination from a physician astute in new and evolving connective tissue diseases such as IgG4-RD.

Serum immunoglobulin G4 (IGG4) is often elevated, but this is not always the case. However, IgG4 blood lab levels greater than 135 are considered an evolving diagnostic criterion for disease suspicion [63]


Treatment

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

An international panel of experts have developed recommendations for the management of IgG4-RD. [4] [6] 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 in order to reduce the side-effects of prolonged glucocorticoid usage. 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.

Maintenance

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 maintenance therapy is prednisolone 2.5–5 mg per day, or use of a steroid-sparing agent instead.

Relapse

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. [5]

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. [64]

Other interventions

When organ involvement causes local mechanical problems, further organ-specific interventions may be necessary. For example, when a tumefactive lesion causes 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.

Trials

Research is also underway to evaluate the effect and safety of plasmablast-directed therapy with a monoclonal antibody (XmAb5871, obexelimab) which inhibits B-cell function without depleting these immune cells. [65]

Epidemiology

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 2.8–10.8/million population, with a median age of onset of 58 years. [66] Another study based on United States healthcare administrative claims data estimated the prevalence of IgG4-RD at 5.3/100,000 (about 1/20,000). [67]

Nomenclature

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. [2] [66] In addition, there are a number of historical reports in the literature of disease associations, which, in retrospect are likely to have been different manifestations of IgG4 disease. For example, Banerjee et al[1989] reported two patients with autoimmune hepatitis and febrile panniculitis. On each occasion the panniculitis responded to increasing the doses ofprednisolone. [68]

At the International Symposium on IgG4-Related Diseases, the consensus name of IgG4-related disease was endorsed for the condition. [2] This name had already been agreed upon as a consensus name among Japanese investigators, [2] [3] 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. [66]

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. [2]

An expanded term, 'Immunoglobulin G4-related disease', has sometimes been used also. [69] However, this term was never referenced in the 2012 recommendations for nomenclature, [2] and its use would appear to be erroneous.

See also

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References

  1. ↑ Wallace, Zachary S.; Deshpande, Vikram; Mattoo, Hamid; Mahajan, Vinay S.; Kulikova, Maria; Pillai, Shiv; Stone, John H. (26 August 2015). "IgG4-Related Disease: Clinical and Laboratory Features in One Hundred Twenty-Five Patients". Arthritis & Rheumatology . 67 (9): 2466–2475. doi:10.1002/art.39205. PMC   4621270 . PMID   25988916.
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  5. 1 2 Terumi Kamisawa; Yoh Zen; Shiv Pillai; John H. Stone (11 April 2015). "IgG4-related disease". The Lancet . 385 (9976): 1460–1471. doi:10.1016/S0140-6736(14)60720-0. PMID   25481618. S2CID   28886899.
  6. 1 2 Walsh, Nancy (1 April 2015). "Experts Weigh In on IgG4 Disease - Diagnosis requires biopsy, and treatment starts with steroids". MedPage Today. Retrieved 6 March 2016.
  7. ↑ Erickson, A; Al-Khalili, O (May–June 2018). "IgG-4 Related Disease: An Introduction". Missouri Medicine. 115 (3): 253–256. PMC   6140155 . PMID   30228732.
  8. 1 2 3 4 Somalinga Nagendran Karthik; K. Bhanu; Sakthi Velayutham; M. Jawahar (July–September 2011). "Hypertrophic pachymeningitis". Annals of Indian Academy of Neurology. 14 (3): 203–204. doi: 10.4103/0972-2327.85896 . PMC   3200046 . PMID   22028536.
  9. ↑ Young-Sub Lee; Hye Won Lee; Ki-Su Park; Seong-Hyun Park; Jeong-Hyun Hwang (October 2014). "Immunoglobulin G4-Related Hypertrophic Pachymeningitis with Skull Involvement". Brain Tumor Research and Treatment. 2 (2): 87–91. doi:10.14791/btrt.2014.2.2.87. PMC   4231624 . PMID   25408931. Archived from the original on 2016-03-05. Retrieved 2015-08-25.
  10. ↑ Jesica Romina Gallo; Alberto Christian Ortiz; Sergio Oscar Paira (September 2014). "IgG4-Related Disease and Sensorineural Hearing Loss". Clinical and Experimental Otorhinolaryngology. 7 (3): 236–237. doi:10.3342/ceo.2014.7.3.236. PMC   4135164 . PMID   25177444.
  11. ↑ Akira Shimatsu; Yutaka Oki; Ichiro Fujisawa; Toshiaki Sano (December 2009). "Pituitary and stalk lesions (infundibulo-hypophysitis) associated with immunoglobulin G4-related systemic disease: an emerging clinical entity". Endocrine Journal . 56 (9): 1033–1041. doi: 10.1507/endocrj.K09E-277 . PMID   19926920.
  12. ↑ Takahira, Masayuki; Azumi, Atsushi (21 November 2013). "Chapter 12: Ophthalmology". In Hisanori Umehara; Kazuichi Okazaki; John H. Stone; Shigeyuki Kawa; Mitsuhiro Kawano (eds.). IgG4-Related Disease. Springer Science & Business Media. pp. 77–84. doi:10.1007/978-4-431-54228-5. ISBN   978-4-431-54227-8.
  13. ↑ Jiwon Seo; In Ji Song; Sak Lee; Hyeon Joo Jeong; Hye Min Kim; Beom Seok Koh; Sung Ha Park (March 2015). "A Case of Constrictive Pericarditis due to Immunoglobulin G4-Related Disease". Korean Circulation Journal. 45 (2): 161–164. doi:10.4070/kcj.2015.45.2.161. PMC   4372983 . PMID   25810739.
  14. 1 2 3 Changho Song; Myoung Ju Koh; Yong-Nam Yoon; Boyoung Joung; Se Hoon Kim (1 September 2013). "IgG4-related sclerosing disease involving the superior vena cava and the atrial septum of the heart". Yonsei Medical Journal . 54 (5): 1285–1288. doi:10.3349/ymj.2013.54.5.1285. PMC   3743176 . PMID   23918583.
  15. ↑ Qi Qian; Kianoush B. Kashani; Dylan V. Miller (10 September 2009). "Ruptured abdominal aortic aneurysm related to IgG4 periaortitis". The New England Journal of Medicine . 361 (11): 1121–1123. doi: 10.1056/NEJMc0905265 . PMID   19741239.
  16. ↑ Satomi Kasashima; Yoh Zen (26 January 2011). "IgG4-related Inflammatory Abdominal Aortic Aneurysm, Spectrum of IgG4-related Chronic Periaortitis". Annals of Vascular Diseases. 3 (3): 182–189. doi:10.3400/avd.sasvp01002. PMC   3595781 . PMID   23555408.
  17. ↑ John H. Stone; Arezou Khosroshahi; Alan Hilgenberg; Amy Spooner; Eric M. Isselbacher; James R. Stone (October 2009). "IgG4-related systemic disease and lymphoplasmacytic aortitis". Arthritis & Rheumatism . 60 (10): 3139–3145. doi: 10.1002/art.24798 . PMID   19790067.
  18. ↑ Andrea Barp; Marny Fedrigo; Filippo Maria Farina; Sandro Lepidi; Francesco Causin; Chiara Castellani; Giacomo Cester; Thiene; Marialuisa Valente; Claudio Baracchini; Annalisa Angelini (January–February 2016). "Carotid aneurism with acute dissection: an unusual case of IgG4-related diseases". Cardiovascular Pathology. 25 (1): 59–62. doi:10.1016/j.carpath.2015.08.006. PMID   26453089.
  19. ↑ Inoue, Dai; Yoshida, Kotaro; Yoneda, Norihide; Ozaki, Kumi; Matsubara, Takashi; Nagai, Keiichi; Okumura, Kenichirou; Toshima, Fumihito; Toyama, Jun (April 2015). "IgG4-Related Disease: Dataset of 235 Consecutive Patients". Medicine . 94 (15): e680. doi:10.1097/MD.0000000000000680. PMC   4602507 . PMID   25881845.
  20. ↑ Guillermo Delgado-García; Sergio Sánchez-Salazar; Erick Rendón-Ramírez; Mario Castro-Medina; Bárbara Sáenz-Ibarra; Álvaro Barboza-Quintana; María Azalea Loredo-Alanis; David Hernández-Barajas; Dionicio Galarza-Delgado (November 2016). "Myocardial ischemia as presenting manifestation of IgG4-related disease: a case-based review". Clinical Rheumatology. 35 (11): 2857–2864. doi:10.1007/s10067-016-3292-z. PMID   27142374. S2CID   19891657.
  21. ↑ Paulo Sampaio Gutierrez; Thiago Schultz; Sheila Aparecida Coelho Siqueira; Luciano de Figueiredo Borges (November–December 2013). "Sudden coronary death due to IgG4-related disease". Cardiovascular Pathology. 22 (6): 505–507. doi:10.1016/j.carpath.2013.05.003. PMID   23830124.
  22. ↑ Brittany J. Holmes; Nikolay G. Delev; Gary R. Pasternack; Marc K. Halushka (12 June 2012). "Novel cause of sudden cardiac death: IgG4-related disease". Circulation . 125 (23): 2956–2957. doi: 10.1161/CIRCULATIONAHA.111.061002 . PMID   22689933.
  23. ↑ Edward M. Kobraei; Tae H. Song; Douglas J. Mathisen; Vikram Deshpande; Eugene J. Mark (October 2013). "Immunoglobulin G4-Related Disease Presenting as an Obstructing Tracheal Mass: Consideration of Surgical Indications". The Annals of Thoracic Surgery . 96 (4): e91–e93. doi: 10.1016/j.athoracsur.2013.04.120 . PMID   24088500.
  24. 1 2 Hirotsugu Ohkubo; Mikinori Miyazaki; Tetsuya Oguri; Atsushi Arakawa; Yoichiro Kobashi; Akio Niimi (June 2015). "A rare case of IgG4-related disease involving the uterus". Rheumatology . 54 (6): 1124–1125. doi: 10.1093/rheumatology/kev024 . PMID   25810424.
  25. ↑ Jong Hyun Choi; Jae Kyeom Sim; Jee Youn Oh; Eun Joo Lee; Gyu Young Hur; Seung Heon Lee; Sung Yong Lee; Je Hyeong Kim; Sang Yeub Lee; Chol Shin; Jae Jeong Shim; Kwang Ho In; Kyung Ho Kang; Kyung Hoon Min (April 2014). "A Case of IgG4-Related Disease Presenting as Massive Pleural Effusion and Thrombophlebitis". Tuberculosis and Respiratory Diseases. 76 (4): 179–183. doi:10.4046/trd.2014.76.4.179. PMC   4021266 . PMID   24851132.
  26. 1 2 Hyuk Lee; Mee Joo; Tae Jun Song; Sun Hee Chang; Hanseong Kim; Yeon Soo Kim; Ji Yoon Ryoo (April 2011). "IgG4-related sclerosing esophagitis: a case report". Gastrointestinal Endoscopy . 73 (4): 834–837. doi:10.1016/j.gie.2010.08.043. PMID   21067736.
  27. 1 2 Myriam Dumas-Campagna; Simon Bouchard; Genevieve Soucy; Mickael Bouina (August 2014). "IgG4-related esophageal disease presenting as esophagitis dissecans superficialis with chronic strictures". Journal of Clinical Medicine Research. 6 (4): 295–298. doi:10.14740/jocmr1845w. PMC   4039102 . PMID   24883156.
  28. ↑ Bruno Coulier; Luc Montfort; Gabriela Beniuga; Frederic Pierard; Isabelle Gielen (January–February 2014). "Small Bowel Obstruction Caused by Peritoneal Immunoglobulin G4-Related Disease Mimicking Carcinomatosis: Case Report". Korean Journal of Radiology. 15 (1): 66–71. doi:10.3348/kjr.2014.15.1.66. PMC   3909864 . PMID   24497794.
  29. 1 2 Yassaman Raissian; Samih H. Nasr; Christopher P. Larsen; Robert B. Colvin; Thomas C. Smyrk; Naoki Takahashi; Ami Bhalodia; Aliyah R. Sohani; Lizhi Zhang; Suresh Chari; Sanjeev Sethi; Mary E. Fidler; Lynn D. Cornell (July 2011). "Diagnosis of IgG4-Related Tubulointerstitial Nephritis". Journal of the American Society of Nephrology. 22 (7): 1343–1352. doi:10.1681/ASN.2011010062. PMC   3137582 . PMID   21719792.
  30. ↑ Satoshi Yazawa; Rei Ohara; Takahiro Maeda; Kent Kanao; Seiya Hattori; Yosuke Nakajima; Mototsugu Oya (July 2013). "[Three cases of IgG4-related disease associated with urinary tract obstruction]". Nihon Hinyokika Gakkai Zasshi (The Japanese Journal of Urology) (in Japanese). 104 (4): 620–625. doi: 10.5980/jpnjurol.104.620 . PMID   23971373.
  31. 1 2 Bobby B Najari; Brian D Robinson; Stephen A Paget; Darius A Paduch (October 2014). "Clinical, radiographic, and pathologic description of IgG4-related perivasal fibrosis: a previously undescribed etiology of chronic orchialgia". Urology . 84 (4): 748–750. doi:10.1016/j.urology.2014.06.035. PMID   25260440.
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  34. 1 2 3 4 Herwig Pieringer; Ilse Parzer; Adelheid Wöhrer; Petra Reis; Bastian Oppl; Jochen Zwerina (16 July 2014). "IgG4- related disease: an orphan disease with many faces". Orphanet Journal of Rare Diseases . 9: 110. doi: 10.1186/s13023-014-0110-z . PMC   4223520 . PMID   25026959.
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  39. ↑ Wah Cheuk; Fiona K Y Tam; Alice N H Chan; Ivy S C Luk; Anthony P W Yuen; Wai-Kong Chan; Terry C W Hung; John K C Chan (November 2010). "Idiopathic cervical fibrosis--a new member of IgG4-related sclerosing diseases: report of 4 cases, 1 complicated by composite lymphoma". The American Journal of Surgical Pathology . 34 (11): 1678–1685. doi:10.1097/PAS.0b013e3181f12c85. PMID   20871392. S2CID   364195.
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  41. ↑ Akiyuki Hiraga; Daisuke Ozaki; Atsuko Tsuneyama; Seiro Ito; Kyosuke Koide; Satoshi Kuwabara (15 October 2015). "Corticosteroid-responsive leptomeningitis with IgG4-positive plasma-cell infiltration". Journal of the Neurological Sciences . 357 (1–2): 338–340. doi:10.1016/j.jns.2015.07.048. PMID   26254623. S2CID   1062402.
  42. ↑ Terumi Kamisawa; Yuyang Tu; Naoto Egawa; Nobuhiro Sakaki; Jun-ichi Ishiwata; Kouji Tsuruta; Atsutake Okamoto; Toshio Takahashi; Morio Koike (January 2001). "[Clinicopathologic study on chronic pancreatitis with diffuse irregular narrowing of the main pancreatic duct]". Nippon Shokakibyo Gakkai Zasshi (The Japanese Journal of Gastroenterology) (in Japanese). 98 (1): 15–24. doi:10.11405/nisshoshi1964.98.15. PMID   11201120.
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  46. ↑ 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: 10.1186/s13000-014-0225-5 . PMC   4265405 . PMID   25487870.
  47. ↑ 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 . 458 (1): 109–113. doi:10.1007/s00428-010-0995-4. PMID   20957491. S2CID   21848271.
  48. ↑ 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 . 223 (4): 301–305. doi:10.1159/000335372. PMID   22269779. S2CID   22928854.
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  50. ↑ 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: 10.1155/2012/401890 . PMC   3317227 . PMID   22523496.
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  52. ↑ 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: 10.5402/2011/873087 . PMC   3168566 . PMID   21991533.
  53. ↑ 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:10.3348/kjr.2013.14.5.776. PMC   3772257 . PMID   24043971.
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  61. ↑ Takao Taniguchi; Hisato Kobayashi; Shouichi Fukui; Keiji Ogura; Tatuyoshi Saiga; Motozumi Okamoto (September 2006). "A case of multifocal fibrosclerosis involving posterior mediastinal fibrosis, retroperitoneal fibrosis, and a left seminal vesicle with elevated serum IgG4". Human Pathology . 37 (9): 1237–1239. doi:10.1016/j.humpath.2006.03.021. PMID   16938531.
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