Polyclonal hyperviscosity syndrome in IgG4‐related disease and associated conditions

Chen, Luke Y C; Wong, P.C.; Noda, Shinichi; Collins, David R.; Sreenivasan, Gayatri M.; Coupland, Robert C.

doi:10.1002/ccr3.201

Cited by 20 publications

(16 citation statements)

References 34 publications

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“…In fact, we previously published a case report with a diagnostic label of idiopathic HES, reviewed by several world experts in eosinophilia who concurred with the diagnosis, which was subsequently found to be IgG4-RD. 27,28 Findings of a myeloid clonal disorder such as increased blast cells, abnormal karyotype, mutations in PDGFR-alpha/beta, FGFR-1 and PCM1-JAK2 are not seen in IgG4-RD. However, differentiating lymphocytic-variant HES from IgG4-RD can be more challenging.…”

Section: Clinical Presentationmentioning

confidence: 99%

IgG4-related disease: what a hematologist needs to know

Mattman²,

et al. 2019

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IgG4-related disease is a fibro-inflammatory condition that can affect nearly any organ system. Common presentations include major salivary and lacrimal gland enlargement, orbital disease, autoimmune pancreatitis, retroperitoneal fibrosis and tubulointerstitial nephritis. This review focuses on the hematologic manifestations of IgG4-related disease, including lymphadenopathy, eosinophilia, and polyclonal hypergammaglobulinemia. The disease can easily be missed by unsuspecting hematologists, as patients may present with clinical problems that mimic disorders such as multicentric Castleman disease, lymphoma, plasma cell neoplasms and hypereosinophilic syndromes. When IgG4-related disease is suspected, serum protein electrophoresis and IgG subclasses are helpful as initial tests but a firm histological diagnosis is essential both to confirm the diagnosis and to rule out mimickers. The central histopathological features are a dense, polyclonal, lymphoplasmacytic infiltrate enriched with IgG4-positive plasma cells (with an IgG4/IgG ratio >40%), storiform fibrosis, and obliterative phlebitis. Importantly for hematologists, the latter two features are seen in all tissues except bone marrow and lymph nodes, making these two sites suboptimal for histological confirmation. Many patients follow an indolent course and respond well to treatment, but a significant proportion may have highly morbid or fatal complications such as periaortitis, severe retroperitoneal fibrosis or pachymeningitis. Corticosteroids are effective but cause new or worsening diabetes in about 40% of patients. Initial response rates to rituximab are high but durable remissions are rare. More intensive lymphoma chemotherapy regimens may be required in rare cases of severe, refractory disease, and targeted therapy against plasmablasts, IgE and other disease biomarkers warrant further exploration.

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Section: Clinical Presentationmentioning

confidence: 99%

IgG4-related disease: what a hematologist needs to know

Mattman²,

et al. 2019

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“…Given that IgG4‐RD and L‐HES present with overlapping clinical and laboratory features such as asthma, atopy, lymphadenopathy, eosinophilia, and elevated serum immunoglobulins, differentiating between these two rare entities is an underappreciated diagnostic challenge . We previously published the case of a young woman with polyclonal hyperviscosity syndrome and eosinophilia labeled as idiopathic HES, a diagnosis confirmed prior to publication by international experts in eosinophilia (case I3 in this study); however, 2 years after publication, she was found to have histologically confirmed IgG4‐RD . Given the potential diagnostic confusion between these two entities, and the lack of large studies comparing them, we conducted a comparative case series describing the clinical and laboratory features of patients with IgG4‐RD and L‐HES.…”

Section: Introductionmentioning

confidence: 67%

IgG4‐related disease and lymphocyte‐variant hypereosinophilic syndrome: A comparative case series

Carruthers

Park

Slack

et al. 2017

European J of Haematology

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“…Steroid-sparing agents such as azathioprine, mycophenolate mofetil, 6-mercaptopurine, methotrexate, tacrolimus, or cyclophosphamide have been used, mainly in the setting of AIP, with limited success [ 38 ]. Rituximab is effective in most cases refractory to steroids and other immunomodulators, although rare cases requiring more intensive therapy such as purine analogues have been reported [ 39 ]. A recent case report confirmed the effectiveness of rituximab in a steroid-refractory patient with IgG4-TIN, with sustained improvement in renal function [ 40 ].…”

Section: Main Textmentioning

confidence: 99%

Recognizing IgG4-Related Tubulointerstitial Nephritis

Mann

Seidman

Barbour

et al. 2016

Can J Kidney Health Dis

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Purpose of the reviewImmunoglobulin G4-related disease (IgG4-RD) is a systemic fibroinflammatory disorder affecting nearly all organs, including the kidney. Tubulointerstitial nephritis (IgG4-TIN) is the most common form of IgG4-related kidney disease (IgG4-RKD) and is the focus of this concise review.ObjectiveThe study aims to describe when IgG4-TIN should be suspected and to summarize the diagnosis, treatment, and natural history of the disease.Sources of informationOvid MEDLINE, Google Scholar, and PubMed were searched for full-text English language articles up to January 2016. References included in the manuscript were chosen at the authors’ discretion based on their relevance to the subject of the review.FindingsIgG4-TIN should be considered in patients presenting with abnormal urinalysis, abnormal kidney function, renal lesions on imaging, and elevated IgG, IgE, or hypocomplementemia. Diagnosis of IgG4-TIN requires a combination of histologic features (plasma cell-enriched TIN with >10 IgG4+ plasma cells/hpf, +/− TBM immune complex deposits in many cases) and at least one of the following:Characteristic radiologic findings (small peripheral low-attenuation cortical nodules, round or wedge-shaped lesions, or diffuse patchy involvement)Elevated serum IgG4 levelCharacteristic findings of IgG4-RD in other organsOther conditions such as lupus, vasculitis, diabetic nephropathy, and lymphoma must be excluded, as these can also present with IgG4+ plasma cells in the renal parenchyma. IgG4-TIN is generally responsive to steroids and B cell depletion with rituximab, but relapses are common and patients require close long-term follow-up.LimitationsAvailable data on IgG4-TIN are from retrospective observational studies.ImplicationsIgG4-TIN is a distinct and emerging subtype of interstitial nephritis. Nephrologists must be aware of this entity and how to definitively diagnose and treat it. Prospective studies and ideally multi-center clinical trials are needed to study the epidemiology, treatment, and natural history of this disease.

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Polyclonal hyperviscosity syndrome in IgG4‐related disease and associated conditions

Cited by 20 publications

References 34 publications

IgG4-related disease: what a hematologist needs to know

IgG4-related disease: what a hematologist needs to know

IgG4‐related disease and lymphocyte‐variant hypereosinophilic syndrome: A comparative case series

Recognizing IgG4-Related Tubulointerstitial Nephritis

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