Isolated Renal Involvement by IG4-Related Disorder Mimicking Multiple Myeloma, a Diagnosis Not to Miss

Khalil, Muhammad Abdul Mabood; Rajput, Ahmed Suleman; Ghani, Ruzita; Ullah, S. M. Rahmat; Thet, May Kyaw; Daiwajna, Rajendra; Telisinghe, Pemasari Upali; Chong, Vui Heng; Tan, Jackson

doi:10.4103/1319-2442.318534

Cited by 2 publications

(5 citation statements)

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“…IgG4-RD, a rare immunologic condition, presents as single or multi-organ/tissue spreading at pulmonary, orbital areas, salivary glands (such as chronic sclerosing sialadenitis, also named Kűttner tumor, and Mikulicz’s disease), biliary ducts and in the gallbladder (sclerosing cholangitis and cholecystitis), renal system (interstitial nephritis), pancreatic system (multifocal autoimmune pancreatitis), and lymph nodes in association with various cardiovascular manifestations (such as aortitis, pericarditis, vasculitis, and pulmonary vascular disease) [ 22 , 23 , 24 , 25 , 26 ]. Virtually, any organ may become a host of the condition [ 27 ].…”

Section: Introductionmentioning

confidence: 99%

“…Genetic susceptibility regarding CFHR 1 and CFHR 4 gene deletions has been suggested [ 25 , 38 ]. The highly specific histologic features include an increased (dense) lymphoplasmacytic infiltration, high content of IgG4-positive plasma cells, storiform fibrosis, and (obliterate) phlebitis [ 22 , 24 ]. Needle biopsy is less likely to point out all these mentioned traits compared with surgical (open) biopsy depending on the organ and disease severity [ 39 ].…”

Section: Introductionmentioning

confidence: 99%

“…In terms of treatment, firstly, patients are candidates for glucocorticoid therapy, which might rapidly improve the clinical evolution in the majority of cases; thus, prompt and adequate disease recognition and intervention is important (if feasible) [ 24 ]. Non-responders are offered second-line medication such as rituximab, as well as other drugs such as cyclophosphamide or mycophenolate mofetil, etc.…”

Section: Introductionmentioning

confidence: 99%

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Reshaping the Concept of Riedel’s Thyroiditis into the Larger Frame of IgG4-Related Disease (Spectrum of IgG4-Related Thyroid Disease)

Cârșote

Nistor

2023

Biomedicines

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Recently, Riedel’s thyroiditis (RT) was assimilated into the larger spectrum of immunoglobulin IgG4-related disease (IgG4-RD) in addition to a particular frame of IgG4-related thyroid disease (IgG4-RTD), underlying IgG4-RT, IgG4-associated Hashimoto’s thyroiditis (and its fibrotic variant), and IgG4-related Graves’s disease. Our objective was to overview recent data on RT, particularly IgG4-RD and IgG4-RTD. The case and study– sample analysis (2019–2023) included 293 articles and selected 18 original studies: nine single case reports (N = 9, female/male = 2/1, aged: 34–79 years, 5/9 patients with serum IgG4 available data, 2/5 with high serum IgG4) and four case series (N = 21; 4/5 series provided data on IgG4 profile, 3/21 had serum IgG4 assays, and 2/3 had abnormally high values). IgG4-RD and thyroid findings were analyzed in three cohorts (N = 25). Another two studies (N = 11) specifically addressed IgG4-RTD components. On presentation, the patients may have hypothyroidism, transitory thyrotoxicosis, goiter, long-term history of positive anti-thyroid antibodies, and hypoechoic ultrasound thyroid pattern. The 5-year analysis (N = 66) showed the rate of serum IgG4 evaluation remained low; normal values do not exclude RT. Mandatory histological and immunohistochemistry reports point out a high content of IgG4-carrying plasma cells and IgG4/IgG ratio. Unless clinically evident, histological confirmation provides a prompt indication of starting corticoid therapy since this is the first-line option. Surgery, if feasible, is selective (non-responders to medical therapy, emergency tracheal intervention, and open/core needle biopsy). Current open issues are identifying the role of serum IgG4 assays in patients with IgG4-RD, finding out if all cases of RT are IgG4-mediated, applying IgG4-RTD criteria of differentiation among four entities, and providing an RT/IgG4-RTD guideline from diagnosis to therapy. It remains that the central aim of approaching RT in daily practice is the early index of suspicion in order to select patients referred for further procedures that provide enough histological/immunohistochemistry material to confirm RT and its high IgG4 burden.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Reshaping the Concept of Riedel’s Thyroiditis into the Larger Frame of IgG4-Related Disease (Spectrum of IgG4-Related Thyroid Disease)

Cârșote

Nistor

2023

Biomedicines

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“…With the increasing number of reports and research in this field, clinicians have gradually become familiar with this syndrome. IgG4-related bone marrow presentation has rarely been mentioned before [ 7 ], since fibrosis and obliterative phlebitis is typically not seen, resulting in insensitivity to diagnosis [ 8 ]. Kamisawa et al .…”

Section: Discussionmentioning

confidence: 99%

“…firstly described histologic feature of IgG4-positive plasma cell infiltration of the BM in two patients with AIP in 2003 [ 9 ]. Moreover, in a case report of isolated IgG4-RKD with kappa light chain deposit, BM biopsy revealed around 20% of plasma cell infiltration without monoclonal paraprotein [ 8 ]. As reported in published studies, BM involvement of IgG4-RD usually presented pancytopenia, accompanied by other hematologic manifestations, including lymphadenopathy, eosinophilia and polyclonal hypergammaglobulinemia [ 6 ], and was finally confirmed by subsequent BM pathology.…”

Section: Discussionmentioning

confidence: 99%

Active IgG4-related disease with bone marrow involvement: a report of 2 cases and case-based review

Liu

Wang

2022

Eur J Med Res

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IgG4-related disease (IgG4-RD) is a systemic fibro-inflammatory disease, histopathologically characterized by dense lymphoplasmacytic infiltration rich in IgG4-positive plasma cells in affected organs. Classic hematologic presentations including lymphadenopathy, eosinophilia and polyclonal hypergammaglobulinemia are common, whereas bone marrow involvement of IgG4-RD is rarely reported. Here we present two patients of multi-organ IgG4-RD with bone marrow involvement, one on bone marrow biopsy, and the other on PET/CT. Presentations of other organ involvement included biopsy-proven IgG4-related tubulointerstitial nephritis, lymphadenopathies, submaxillary glands, arteritis, asthma, dysosmia, and constitutional symptoms. Bone marrow involvement was initially suspected due to leukopenia, anemia and thrombocytopenia in case#1, and was finally confirmed by histological evidence of significant IgG4-positive plasma cells infiltration in bone marrow. In case#2, we incidentally observed high uptakes of multi-bone marrow on 18F-FDG-PET/CT, with the maximum SUV value similar to that of the kidneys, submaxillary glands and hilar, mediastinal lymph nodes by 18F-FDG-PET/CT. Symptoms and all the hematologic presentation improved rapidly in both patients after steroids initiation. These two cases illustrate the rare bone marrow involvement in active IgG4-RD accompanied by other hematologic syndromes. The significance of disease pathogenesis is worthy of further study.

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Isolated Renal Involvement by IG4-Related Disorder Mimicking Multiple Myeloma, a Diagnosis Not to Miss

Cited by 2 publications

References 0 publications

Reshaping the Concept of Riedel’s Thyroiditis into the Larger Frame of IgG4-Related Disease (Spectrum of IgG4-Related Thyroid Disease)

Reshaping the Concept of Riedel’s Thyroiditis into the Larger Frame of IgG4-Related Disease (Spectrum of IgG4-Related Thyroid Disease)

Active IgG4-related disease with bone marrow involvement: a report of 2 cases and case-based review

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