Case 1‑2012: ANCA Associated Glomerulonephritis in Combination with IgG4 Positive Mediastinal Mass in a Patient with Ankylosing Spondylitis Treated with TNF Alpha Inhibitors

Tošovský, M.; Bradna, P; Laco, Ján; Podhola, Miroslav; Soukup, Tomáš; Brožík, Jan

doi:10.14712/18059694.2015.74

Cited by 19 publications

(9 citation statements)

References 13 publications

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“…[9] Two patients were diagnosed with AAV in the absence of specific findings to diagnose GPA, MPA, or EGPA definitively. [6,7] One patient was diagnosed with GPA (present case).…”

Section: Review Of the Literaturementioning

confidence: 84%

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Antineutrophil cytoplasmic antibody positivity in IgG4-related disease

et al. 2016

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Background:IgG4-related disease (IgG4-RD) is a fibroinflammatory condition characterized by serum IgG4 elevation and tissue infiltration of IgG4-positive plasma cells. Substantial overlap between IgG4-RD and antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitides (AAV) exists in terms of organ involvement and histopathological features. A positive ANCA assay is regarded as a highly specific finding in favor of an AAV, and generally influences away from a diagnosis of IgG4-RD. Recent reports, however, have raised the possibility that some patients with IgG4-RD are ANCA positive, thus suggesting reconsideration of the role of ANCA in the diagnostic workup. In the present work, we describe the first case of concomitant biopsy-proven IgG4-RD and granulomatosis with polyangiitis (GPA), demonstrating antiproteinase 3 (PR3) ANCA of the IgG4 subclass in the patient's serum. We also review the literature in order to provide clinicians with tools for interpreting ANCA positivity in IgG4-RD patients.Case summary:A 51-year-old woman was referred for left exopthalmos due to lacrimal gland enlargement and increased serum IgG4 concentration. IgG4-RD was suspected and further imaging studies disclosed multiple pulmonary masses in the right lung. Histological analysis of the left lacrimal gland was diagnostic for IgG4-RD, but lung biopsy showed typical features of GPA. ANCA assay was positive for anti-PR3 antibodies. Further immunofluorescence studies demonstrated anti-PR3 antibodies of IgG1 and IgG4 subclass. Treatment with rituximab induced swift remission of both IgG4-RD and GPA manifestations. We identified 9 other reports of patients with IgG4-RD and positive ANCA in the English literature, 5 cases with biopsy-proven IgG4-RD and 4 cases in whom IgG4-RD was diagnosed presumptively. Four patients had also histological evidence of concomitant AAV.Conclusion:The present work demonstrates that ANCA positivity in patients with biopsy-proven IgG4-RD should prompt the exclusion of a concomitant vasculitic process; a positive ANCA does not exclude the diagnosis of IgG4-RD; confirmation through immunoenzymatic assays of the ANCA specificity, clinical-pathological correlation, and histopathological evaluation remain crucial steps for the differential diagnosis between AAV and IgG4-RD.

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“…[9] Two patients were diagnosed with AAV in the absence of specific findings to diagnose GPA, MPA, or EGPA definitively. [6,7] One patient was diagnosed with GPA (present case).…”

Section: Review Of the Literaturementioning

confidence: 84%

“…Similarly, intravenous cyclophosphamide and methylprednisolone pulses were required in addition to oral prednisone in order to prevent renal failure in a patient with rapidly progressive glomerulonephritis. [7] …”

Section: Review Of the Literaturementioning

confidence: 99%

Antineutrophil cytoplasmic antibody positivity in IgG4-related disease

et al. 2016

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“…Another prospective series of patients with ankylosing spondylitis did not show a significant increase in ANCA seroconversion in treated patients (52). Six patients with TNF-a inhibitor AAV have been reported to date (53,54).…”

Section: Drugs Associated With Aavmentioning

confidence: 99%

Drug-Induced Glomerular Disease

Hogan

Markowitz²,

Radhakrishnan

2015

Clinical Journal of the American Society of Nephrology

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Drug-induced autoimmune disease was initially described decades ago, with reports of vasculitis and a lupus-like syndrome in patients taking hydralazine, procainamide, and sulfadiazine. Over the years, multiple other agents have been linked to immune-mediated glomerular disease, often with associated autoantibody formation. Certain clinical and laboratory features may distinguish these entities from their idiopathic counterparts, and making this distinction is important in the diagnosis and management of these patients. Here, drug-induced, ANCA-associated vasculitis, drug-induced lupus, and drug-associated membranous nephropathy are reviewed.

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“…However, we are unable to determine whether all cases fulfilled the comprehensive diagnostic criteria for IgG4-related disease, as the number of IgG4+ plasma cells / HPF and the ratio of IgG4+ / IgG+ plasma cells were not shown in some cases. Four of the five cases received only steroid therapy and responded to treatment, while the other case received steroid and intravenous cyclophosphamide therapy because he had ANCA-associated glomerulonephritis (21). Our patient needed immunosuppressants with steroids because of his frequent relapses.…”

Section: Discussionmentioning

confidence: 82%

“…Only five cases of IgG4-related disease or multifocal fibrosclerosis with a soft tissue mass around the thoracic vertebral bodies have been previously reported (19)(20)(21)(22)(23). The clinical and demographic characteristics of the five cases are shown in Table. All five patients are men with an age range of 40 to 70 years.…”

Section: Discussionmentioning

confidence: 99%

Multifocal Fibrosclerosis with Hypertrophic Pachymeningitis and a Soft Tissue Mass around the Thoracic Vertebral Bodies: A Case Report with Review of the Literature

et al. 2015

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Multifocal fibrosclerosis is the term used to represent a combination of similar fibrous lesions occurring at different anatomical sites. We herein report a hypertrophic pachymeningitis patient with a soft tissue mass around the thoracic vertebral bodies. A histopathological analysis of the biopsied tissues from both lesions showed dense fibrosis and a marked infiltration of lymphocytes and plasma cells, which lead to the diagnosis of multifocal fibrosclerosis. This pathological condition closely resembles that of IgG4-related disease and is a very rare combination of manifestations. Our case suggests that hypertrophic pachymeningitis patients need to also undergo a whole body examination.

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Case 1‑2012: ANCA Associated Glomerulonephritis in Combination with IgG4 Positive Mediastinal Mass in a Patient with Ankylosing Spondylitis Treated with TNF Alpha Inhibitors

Cited by 19 publications

References 13 publications

Antineutrophil cytoplasmic antibody positivity in IgG4-related disease

Antineutrophil cytoplasmic antibody positivity in IgG4-related disease

Drug-Induced Glomerular Disease

Multifocal Fibrosclerosis with Hypertrophic Pachymeningitis and a Soft Tissue Mass around the Thoracic Vertebral Bodies: A Case Report with Review of the Literature

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