“…Polyclonal increase in IgG4 has been described in allergic disorders , parasitic infections , and multicentric Castleman's disease among other conditions. Reactive polyclonal hypergammaglobulinemia is seen in many inflammatory conditions and can rarely lead to hyperviscosity in HIV , hypereosinophilic syndrome , rheumatoid arthritis , and Sjögren's syndrome . One other patient with hyperviscosity syndrome, lymphadenopathy, and elevated serum IgG4 has been reported , but to our knowledge was not confirmed to have IgG4‐RD.…”
Immunoglobulin G4-related disease (IgG4-RD) is a recently described entity with protean manifestations. We describe a novel case of IgG4-RD with hypergammaglobulinemic hyperviscosity responsive to fludarabine and rituximab. A 33-year-old Asian man developed bilateral lacrimal gland and submandibular salivary gland swelling with cervical lymphadenopathy. Biopsies of the affected tissues revealed reactive follicular hyperplasia. Seven years later, he presented with bilateral retinal hemorrhages due to hyperviscosity syndrome from profound polyclonal increase in IgG, including marked IgG4 elevation. Despite plasmapheresis, overproduction of IgG continued and he was refractory to systemic steroids, azathioprine, interferon alpha, and cyclophosphamide. IgG4-RD was suspected following a myocardial infarction and detection of aneurysmal coronary arteries indicating large vessel vasculitis. Review of the cervical lymph node and lacrimal gland biopsies with immunohistochemical staining for IgG4-positive plasma cells confirmed IgG4-RD. B-cell depletion with rituximab produced a partial response, but clinical symptoms and elevated protein levels persisted. Fludarabine was added to rituximab to suppress T-cell activity, and this resulted in an excellent clinical and biochemical response. Combination therapy with fludarabine and rituximab in IgG4-RD has not previously been reported and can be considered in patients with severe refractory disease.
“…Polyclonal increase in IgG4 has been described in allergic disorders , parasitic infections , and multicentric Castleman's disease among other conditions. Reactive polyclonal hypergammaglobulinemia is seen in many inflammatory conditions and can rarely lead to hyperviscosity in HIV , hypereosinophilic syndrome , rheumatoid arthritis , and Sjögren's syndrome . One other patient with hyperviscosity syndrome, lymphadenopathy, and elevated serum IgG4 has been reported , but to our knowledge was not confirmed to have IgG4‐RD.…”
Immunoglobulin G4-related disease (IgG4-RD) is a recently described entity with protean manifestations. We describe a novel case of IgG4-RD with hypergammaglobulinemic hyperviscosity responsive to fludarabine and rituximab. A 33-year-old Asian man developed bilateral lacrimal gland and submandibular salivary gland swelling with cervical lymphadenopathy. Biopsies of the affected tissues revealed reactive follicular hyperplasia. Seven years later, he presented with bilateral retinal hemorrhages due to hyperviscosity syndrome from profound polyclonal increase in IgG, including marked IgG4 elevation. Despite plasmapheresis, overproduction of IgG continued and he was refractory to systemic steroids, azathioprine, interferon alpha, and cyclophosphamide. IgG4-RD was suspected following a myocardial infarction and detection of aneurysmal coronary arteries indicating large vessel vasculitis. Review of the cervical lymph node and lacrimal gland biopsies with immunohistochemical staining for IgG4-positive plasma cells confirmed IgG4-RD. B-cell depletion with rituximab produced a partial response, but clinical symptoms and elevated protein levels persisted. Fludarabine was added to rituximab to suppress T-cell activity, and this resulted in an excellent clinical and biochemical response. Combination therapy with fludarabine and rituximab in IgG4-RD has not previously been reported and can be considered in patients with severe refractory disease.
“…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.…”
“…This report updates his unique clinical course and ongoing therapy, including recent response to bendamustine therapy. Case 2 had been previously diagnosed with idiopathic hypereosinophilic syndrome when her case was published in 2010 17 , but a pathology review in 2013 demonstrated that she in fact had IgG4-RD. Case 3 is a case of systemic plasmacytosis with hyperviscosity 18 , and the laboratory and pathology findings pertaining to IgG4-RD are provided here.…”
Section: Methodsmentioning
confidence: 99%
“…This woman presented at age 21 with HVS, episodic angioedema, lymphadenopathy, and eosinophilia, which, after extensive investigation, was thought to be due to idiopathic hypereosinophilic syndrome (HES) 17 . Of note, her case had been reviewed by numerous clinicians and pathologists who are world experts in eosinophilia and lymphoproliferative disorders before this diagnosis was agreed upon.…”
Key Clinical MessagePolyclonal hyperviscosity syndrome (HVS) is rare and has been reported in various disorders of immune dysregulation and lymphoid hyperplasia. IgG4-Related Disease (IgG4-RD) is an emerging disorder often associated with exuberant hypergammaglobulinemia, and this review of seven cases establishes IgG4-RD as an important cause of polyclonal HVS.
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