Churg-Strauss Syndrome Concomitant with Chronic Symmetrical Dacryoadenitis Suggesting Mikulicz's Disease

Hanioka, Yusuke; Yamagami, Keiko; Yoshioka, Katsunobu; Nakamura, Tomomi; Kishida, Masatsugu; Nakamura, Tomoyuki; Yamaguchi, Toshimasa; Koshimo, Naomi; Inoue, Takeshi; Imanishi, Masahito

doi:10.2169/internalmedicine.51.7725

Cited by 34 publications

(13 citation statements)

References 12 publications

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“…Paulus at al [10] achieved prompt clinical remission in a patient with scleritis and orbital myositis by combining methotrexate and intraocular triamcinolone to oral prednisone. In the case reported by Hanioka et al, [9] prednisolone led to a reduction of lacrimal gland swelling, but peripheral neuropathy improved only with intravenous immunoglobulins and methyprednisolone pulses. 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.…”

Section: Review Of the Literaturementioning

confidence: 99%

“…Among these 4 patients, 1 was diagnosed with EGPA based on a history of long-standing asthma, multiple mononeuritis, peripheral blood eosinophilia, ANCA positivity with a specificity for MPO, and histological evidence of cutaneous leukocytoclastic vasculitis with abundant eosinophils. [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: 99%

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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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Section: Review Of the Literaturementioning

confidence: 99%

Section: Review Of the Literaturementioning

confidence: 99%

Antineutrophil cytoplasmic antibody positivity in IgG4-related disease

et al. 2016

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“…In the cases with diagnosis of GPA, increased IgG4-positive plasma cell in orbital biopsies was reported [2, 6]. There are some reports about CSS concomitant with clinical conditions similar to IgG4-RD [7, 8]. However, reports about the orbital manifestations similar to IgG4-RD in EGPA were relatively rare [9].…”

Section: Discussionmentioning

confidence: 99%

A Case of Eosinophilic Granulomatosis with Polyangiitis Complicated with A IgG4 Related Disease Like Symptoms

Sato

Morimoto

Oguchi

et al. 2018

Case Reports in Immunology

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We report a case of eosinophilic granulomatosis with polyangiitis (EGPA) complicated with a IgG4 related disease like symptoms presenting as eyelid swellings. In the present case, the serum level of IgG4 and the ratio of IgG4 to IgG were generally increased by the disease course of EGPA. Considering the course of clinical symptoms, there is a possibility that orbital manifestations were one of the clinical features during the disease course of EGPA while the histological features of right eyelid tissue and other ocular manifestations were consistent with the diagnosis of IgG4 related disease.

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“…In fact, in the first cases reported by Gambari and Boin (4,5), the swelling of the salivary glands was interpreted as a feature of primary vasculitic involvement. More recently, two cases of patients with EGPA as well as features of Mikulicz's disease (MD) have been reported (6,10).…”

Section: Discussionmentioning

confidence: 99%

Eosinophilic Granulomatosis with Polyangiitis of the Major Salivary Glands: A Case of Sialadenitis in a Young Patient

et al. 2013

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A 23-year-old man was referred to our Emergency Department due to an acute-onset hemoptysis and the associated bilateral swelling of all of his major salivary glands. The elevated levels of systemic inflammatory markers with hypereosinophilia and the concurrent presence of multiple lung infiltrates made it difficult to perform a differential diagnosis, as these symptoms can also be indicative of infectious, autoimmune or hematologic disorders. A histological examination of the patient's left submandibular gland revealed strong clues as to the final diagnosis of eosinophilic granulomatosis with polyangiitis with an atypical clinical presentation, thus allowing for the administration of early and successful conservative therapy. The outcome of our case suggests that systemic vasculitis represents a rare but possible cause of acute bilateral sialadenitis in young patients.

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Churg-Strauss Syndrome Concomitant with Chronic Symmetrical Dacryoadenitis Suggesting Mikulicz's Disease

Cited by 34 publications

References 12 publications

Antineutrophil cytoplasmic antibody positivity in IgG4-related disease

Antineutrophil cytoplasmic antibody positivity in IgG4-related disease

A Case of Eosinophilic Granulomatosis with Polyangiitis Complicated with A IgG4 Related Disease Like Symptoms

Eosinophilic Granulomatosis with Polyangiitis of the Major Salivary Glands: A Case of Sialadenitis in a Young Patient

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