Proposal for diagnostic criteria for IgG4-related kidney disease

Kawano, Mitsuhiro; Saeki, Takako; Nakashima, Hitoshi; Nishi, Shinichi; Yamaguchi, Yutaka; Hisano, Satoshi; Yamanaka, Nobuaki; Inoue, Dai; Yamamoto, Motohisa; Takahashi, Hiroki; Nomura, Hideki; Taguchi, Takashi; Umehara, Hisanori; Makino, Hírofumi; Saito, Takao

doi:10.1007/s10157-011-0521-2

Cited by 384 publications

(445 citation statements)

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“…Our case showed multiple poorly contrasted areas in the renal parenchyma and diffuse kidney enlargement. Also, about 30 % of the patients diagnosed with IgG4-related kidney disease show no abnormality on abdominal CT, with the percentage of patients showing abnormalities having been reported to be 72.1 % by Saeki et al [10] and 70.7 % by Kawano et al [5]. However, many patients do not undergo contrast-enhanced CT, and contrast-enhanced CT is considered necessary as long as the renal function tolerates the modality.…”

Section: Discussionmentioning

confidence: 99%

“…A diagnosis of IgG4-related kidney disease was made, because a high blood IgG4 level, many poorly contrasted areas observed in both kidneys, renal biopsy findings consistent with tubulointerstitial nephritis, and the IgG4-positive plasma cell rate of 89 % fulfilled the Comprehensive diagnostic criteria for IgG4-related disease, 2011 [4]. However, we classified our case as probable in the diagnostic criteria of the Japanese Society of Nephrology [5]. In this case, we could not find the typical storiform fibrosis, because we started steroid therapy early before fibrosis became strong.…”

Section: Introductionmentioning

confidence: 86%

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A case of IgG4-related kidney disease complicated by eosinophilic lung disease

et al. 2014

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IgG4-related disease is a systemic chronic inflammatory disorder characterized by a high blood level of IgG4 and the organ injuries by marked infiltration of IgG4-positive plasma cells and fibrosis. A 71-year-old male was hospitalized for a cough, malaise and anorexia. IgG4-related disease was suspected due to marked elevation of the serum IgG4 level. However, on lung biopsy, only eosinophil infiltration was demonstrated with no plasma cell infiltration. Otherwise abdominal contrastenhanced CT showed mild enlargement of the bilateral kidneys and many differed contrasted areas and FDG PET-CT. Moreover, renal biopsy specimens showed typical tubulointerstitial nephritis with a large number of IgG4-positive plasma cells infiltration (the IgG4/IgG-positive cell rate, 89 %) and fibrosis. We diagnosed this patient as typical IgG4-related kidney disease. He was treated by the moderate dose of prednisolone (0.8 mg/kg/day) alone, and showed prompt response in the clinical condition, and both the lung and kidney lesions. In this case, it was useful for diagnosis of IgG4-related diseases to evaluate an image such as abdominal contrast-enhanced CT and FDG PET-CT. Our case might be one of the possible patterns of IgG4-related lung diseases. In addition, we thought that there might be an association between hypereosinophilia and IgG4-related kidney disease.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 86%

A case of IgG4-related kidney disease complicated by eosinophilic lung disease

et al. 2014

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“…Organ-specific criteria have also been suggested for specialists (43,44). The diagnosis of IgG4-RD should be made by the integration of clinical features, laboratory findings, typical histopathology, and radiology data (Fig 1).…”

Section: Clinical Symptomsmentioning

confidence: 99%

“…IgG4-related kidney disease (IgG4-RKD) typically becomes apparent when renal dysfunction manifests or is detected accidentally by imaging showing multiple clearly demarcated lowdensity lesions, renal swelling, or thickening of renal pelvis wall (43). In some cases, enhanced CT or MRI may detect a "rim sign" of soft tissue around the kidney, like in type 1 AIP (58).…”

Section: Kidneymentioning

confidence: 99%

IgG4-related disease: A relatively new concept for clinicians

Vasaitis

2016

European Journal of Internal Medicine

102

129

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IgG4-related disease (IgG4-RD) is a recently recognized chronic fibrotic inflammation, which can affect almost every organ, and may come to clinical attention first due to visible organ swelling or organ dysfunction, or is identified incidentally by imaging and specific biopsy.The disorder has an allergic background and is immune-mediated. Up-regulated responses of T helper 2 and T regulatory cells and their cytokines play a major role in disease progression.About 30-50% of patients are atopic or have mild eosinophilia. IgG4-RD predominantly affects middle-aged male patients. The cornerstones of diagnosis of the disease are compatible clinical features and typical histopathology. Swelling of salivary and lacrimal glands, lymphadenopathy, and type 1 autoimmune pancreatitis (AIP) are the most common manifestations of the disease. However, other tissues and organs, such as retroperitoneum, lung, kidney, aorta, upper airways, thyroid gland, meninges, heart, mesenterium and skin may be involved. Typical histopathology is lymphoplasmacytic infiltration abundant in IgG4-positive plasma cells, storiform-type fibrosis, and obliterative phlebitis. Elevated serum IgG4 concentration supports the diagnosis. Characteristic imaging features such as a "capsule-like rim" surrounding the pancreatic lesions is highly specific to type 1 AIP. 18F-fluorodeoxyglucose positron emission tomography/computed tomography enables mapping the sites of inflammation, permits evaluation of the extent of the disease, helps in guiding biopsy decision, and may be used in monitoring response to treatment. Glucocorticoids alone or in combination with B-cell depletion with rituximab induces prompt clinical response to IgG4-RD.This article reviews the current understanding, different clinical manifestations, and approaches to diagnosis and treatment of IgG4-RD. 3Highlights Ø IgG4-related disease is a chronic fibrotic inflammation.Ø The disease affects multiple organs, is progressive, and impairs function.Ø The disorder has an allergic background and is immune-mediated.Ø Compatible clinical signs and typical pathology are the basis for the diagnosis.Ø Glucocorticoids and/or B-cell depletion induces prompt clinical response.Immunoglobulin G4-related disease (IgG4-RD) is a chronic fibrotic inflammation, characterized by tissue infiltration by lymphocytes, IgG4-positive plasma cells, simultaneous development of fibrosis, and often, but not always, elevated serum levels of IgG4 (1, 2). The disease can affect multiple organs. Early diagnosis of IgG4-RD is not easy because of the indolent course of the disease with vague, nonspecific symptoms. It may first come to clinical attention as visible organ swelling or detected organ dysfunction due to fibrosis, or is identified incidentally by imaging and specific biopsy when the patient undergoes investigation of suspected tumor disease. Most forms of organ involvement in IgG4-RD, even in the advanced fibrotic stages, respond well to glucocorticoids (3, 4). 4A comprehensive search for all articles in E...

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“…6c). We, therefore, diagnosed him as having the following: definite IgG4-related kidney disease (IgG4-RKD), according to the diagnostic criteria of IgG4-RKD proposed by Kawano et al (2011), probable lung complication of IgG4-RD, and possible submandibular glands and hilar and mediastinal lymph node involvements of IgG4-RD, according to the Japanese comprehensive diagnostic criteria for IgG4-RD (Umehara et al 2012).…”

Section: Case Presentationmentioning

confidence: 99%

Corticosteroid Therapy for a Patient with Relapsing Polychondritis Complicated by IgG4-Related Disease

Yamasue

Nureki

Matsumoto

et al. 2016

Tohoku J. Exp. Med.

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Relapsing polychondritis (RP) is a rare systemic disorder characterized by recurrent, widespread chondritis of the auricular, nasal, and tracheal cartilages. IgG4-related disease (IgG4-RD) is a systemic immunemediated disease characterized by the infiltration of IgG4-bearing plasma cells into systemic organs. Although 25% to 35% of patients with RP have a concurrent autoimmune disease, coexistence of RP and IgG4-RD is rare. We herein report a case of RP complicated by IgG4-RD. A 63-year-old man developed recurrent bilateral ear pain and swelling, recurrent blurred and decreased vision, and migratory multiple joint pain, sequentially within one year. Fourteen months after the first symptom, he experienced dry cough and dyspnea with exertion. A computed tomography (CT) scan detected interstitial pneumonia, swelling of bilateral submandibular glands, bilateral hilar and mediastinal lymphadenopathy, and several nodules in bilateral kidneys. His serum levels of IgG and IgG4 were elevated. The biopsy specimen of auricular cartilage showed infiltrations of inflammatory cells and fibrosis consistent with RP. The IgG4-positive cells were not observed in auricular cartilage. The patient met the diagnostic criteria of RP, including bilateral auricular chondritis, conjunctivitis, iritis and polyarthritis. The biopsy specimens of lung and kidney revealed the significant infiltrations of IgG4-positive plasma cells and fibrosis. We also diagnosed him as having IgG4-RD, affecting bilateral submandibular glands, hilar and mediastinal lymph nodes, lungs, and kidneys. Thus, RP preceded the onset of IgG4-RD. Corticosteroid therapy improved the symptoms and CT scan findings. In conclusion, RP and IgG4-RD do coexist; however, the pathogenesis of their coexistence is unknown.

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Proposal for diagnostic criteria for IgG4-related kidney disease

Abstract: The provisional criteria and algorithm appear to be useful for clarifying the entity of IgG4-RKD and seeking underlying IgG4-RKD cases; however, further experience is needed to confirm the validity of these criteria.

Cited by 384 publications

References 45 publications

A case of IgG4-related kidney disease complicated by eosinophilic lung disease

A case of IgG4-related kidney disease complicated by eosinophilic lung disease

IgG4-related disease: A relatively new concept for clinicians

Corticosteroid Therapy for a Patient with Relapsing Polychondritis Complicated by IgG4-Related Disease

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