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Case Reports
. 2020 Mar 30;8(4):e00552.
doi: 10.1002/rcr2.552. eCollection 2020 May.

Eosinophilic Granulomatous Polyangiitis With IgG4 Hypergammaglobulinaemia and Salivary Gland Swelling

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Free PMC article
Case Reports

Eosinophilic Granulomatous Polyangiitis With IgG4 Hypergammaglobulinaemia and Salivary Gland Swelling

Koichiro Takahashi et al. Respirol Case Rep. .
Free PMC article

Abstract

A 51-year-old woman was admitted to our hospital for cough, fever, purpura in the legs, and salivary gland swelling. Six years ago, she had been diagnosed with bronchial asthma and was treated with a combination of inhaled corticosteroid and long-acting beta2-agonist. Blood examination showed increased eosinophils at 3027 cells/μL and elevated levels of immunoglobulin (Ig) G4 at 261 mg/dL and C-reactive protein at 2.76 mg/dL. Chest radiograph and computed tomography (CT) showed infiltrates in the bilateral lower lobes. Neck CT showed bilateral salivary gland swelling. Pathological examinations of the lungs and skin purpura showed granuloma with eosinophilic infiltration and perivascular dermatitis, respectively. She was diagnosed with eosinophilic granulomatous polyangiitis (EGPA) and treated with corticosteroids, which resolved the eosinophilia, salivary gland swelling, elevated IgG4 titre, and lung infiltration. As our patient did not meet the American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) 2019 criteria of IgG4-related disease, the diagnostic was EGPA with IgG4 hypergammaglobulinaemia and salivary gland swelling.

Keywords: Eosinophilic granulomatous polyangiitis; IgG4; salivary gland.

Figures

Figure 1
Figure 1
Chest radiography and computed tomography (CT) findings in a 51‐year‐old woman with eosinophilic granulomatous polyangiitis (EGPA). (A) Chest radiography shows infiltrates in the bilateral lower lung fields. (B, C) Chest CT shows dense infiltrations in the bilateral lower lobe and bilateral pleural effusion. (D) Head CT shows marked swelling of the bilateral submandibular glands.
Figure 2
Figure 2
Pathological findings in a 51‐year‐old woman with eosinophilic granulomatous polyangiitis. (A, B) In the lungs, there are inflammatory granulations with marked eosinophil infiltration (haematoxylin–eosin stain: A, 40×; B, 100×). (C, D) In the skin, there is eosinophilic infiltration and perivascular dermatitis (haematoxylin–eosin stain: C, 100×; D, 100×).

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