Recurrent Pleural and Pericardium Effusions in a White Woman With IgG4-related Syndrome

Rossi, Giulio; Marchioni, Alessandro; Guicciardi, Novella; Cadioli, Annamaria; Cavazza, Alberto

doi:10.1097/pas.0b013e31819841df

Cited by 29 publications

(19 citation statements)

References 5 publications

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“…Six cases of IgG4-related pleuritis have been reported to date (Table) (6)(7)(8)(9)(10)(11). Although 5 of these patients had multi-organ diseases, one patient had pleuritis only.…”

Section: Discussionmentioning

confidence: 99%

IgG4-related Pleuritis with Chylothorax

Kato¹,

Takayanagi²,

Ishiguro³

et al. 2014

Intern. Med.

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Presently, 6 cases of IgG4-related pleuritis have been reported. We encountered a patient who developed chylothorax due to IgG4-related disease. To our knowledge, such patients have not been reported. This patient developed right-sided chylothorax and left-sided non-chylothorax lymphocyte-predominant pleuritis. Elevated serum and pleural IgG4 concentrations and histopathological analysis of pleural biopsy confirmed the diagnosis of IgG4-related pleuritis. Left-sided pleuritis improved with corticosteroid therapy, but right-sided chylothorax persists. IgG4-related disease can be one cause of chylothorax. Case ReportA 69-year-old man presented to our hospital complaining of cough and dyspnea on exertion in 2003. He had been diagnosed as having bronchial asthma based on his symptoms and change in peak expiratory flow (morning, 180 mL/s; evening, 420 mL/s). He had also undergone an operation for sinusitis. Infiltration of plasma cells, lymphocytes, and eosinophils was found on pathologic examination.Chest X-ray on his initial presentation showed bronchial wall thickening (Fig. 1A), and computed tomography showed bilateral centrilobular nodules (Fig. 1B). Total cell count in the bronchoalveolar lavage fluid in the right middle lobe was 1,500/mm 3 (neutrophils, 52%; lymphocytes, 4%; eosinophils, 33%; macrophages, 11%). Surgical lung biopsy of the lateral basal segment of the left lower lobe showed bronchiolitis with many plasma cells, lymphocytes, and foamy cells in the bronchial wall, leading to a diagnosis of diffuse panbronchiolitis. After administration of fluticasone/ salmeterol and clarithromycin was started, his symptoms improved. However, in July 2011, dyspnea developed, with a percutaneous oxygen saturation on room air of 94% and bilateral pleural effusions ( Fig. 2A). He was admitted to our hospital for further evaluation.On admission, no superficial lymph node or salivary gland swelling was noted. His white blood cell count was 5,800/mm 3 (eosinophils, 6.1%), and serum IgG, IgG4, and IgE were 1,539 mg/dL, 277 mg/dL (normal range, 4-108 mg/dL), and 2,641 IU/mL, respectively. Serum IL-6 was <8 pg/mL, and his serum angiotensin converting enzyme value was within normal range. Autoantibodies were negative. Computed tomography showed bilateral pleural effusions, bronchiectasis with bronchial wall thickening, and centrilobular nodules (Fig. 2B). The right pleural effusion appeared as a white turbid fluid with a total cell count of 3,980/mm 3 (lymphocytes, 92.0%), total protein of 6.1 g/dL, cholesterol of 92 mg/dL, triglyceride of 215 mg/dL, and adenosine deaminase of 40.8 U/L, with no malignant cells. He was diagnosed as having chylothorax according to the established criteria (1). The left pleural effusion appeared as a clear yellow fluid with a total cell count of 5,870/mm 3 (lymphocytes, 88.5%), total protein of 6.3 g/dL, cholesterol of 79 mg/dL, triglyceride of 8 mg/dL, and adenosine deaminase of 39.9 U/L, with no malignant cells. The IgG4 concentrations in the right and left pleural effusions were 57...

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“…Six cases of IgG4-related pleuritis have been reported to date (Table) (6)(7)(8)(9)(10)(11). Although 5 of these patients had multi-organ diseases, one patient had pleuritis only.…”

Section: Discussionmentioning

confidence: 99%

IgG4-related Pleuritis with Chylothorax

Kato¹,

Takayanagi²,

Ishiguro³

et al. 2014

Intern. Med.

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“…This indicates that not only the pathological but also the clinical presentation of IgG4-RD may be distinct among the tissues involved. Accordingly, IgG4-related pericardial disease is mostly characterized by fibrosis of the thickened pericardium and clinical presentation of constrictive pericarditis; however, one report has described acute inflammatory pathology in the pericardium (4)(5)(6)(7)(8)(9)(10)(11)(12)(13).…”

Section: Introductionmentioning

confidence: 99%

Elevated Serum IgG4 Complicated by Pericardial Involvement with a Patchy <sup>18</sup>F-FDG Uptake in PET/CT: Atypical Presentation of IgG4-related Disease

et al. 2015

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IgG4-related pericardial involvement has rarely been reported and its clinical features remain unknown. We herein report a case of a 50-year-old woman with pericarditis who presented with a fever, elevated C-reactive protein levels, elevated serum IgG4 concentrations, and thickened pericardium with a patchy 18 Ffluorodeoxyglucose (FDG) uptake. A biopsy specimen of 18 F-FDG accumulated in the mediastinal lymph nodes revealed an abundant infiltration of IgG4-bearing plasma cells without fibrosis. Moderate-dose glucocorticoids promptly resolved the physical, serological, and imaging abnormalities, thus indicating a relatively acute and reversible nature of IgG4-related pericardial involvement.

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“…According to their report, imaging disclosed groundglass attenuation and honeycombing, and a transbronchial lung biopsy showed alveolar IgG4+ cell infiltrates. Since then, not only IP, but also a wide variety of other disease states, such as organising pneumonia (OP) (29), inflammatory pseudotumour (IPT) (30), lymphomatoid granulomatosis-like nodules (31), mediastinal lymphadenopathy (32) and involvement of pleural effusions (33), have become generally recognised to develop concurrently with AIP. Zen et al (34) summarised nine cases of IPT, showing that marked IgG4+ cell infiltration was characteristic of plasma-cell type IPT.…”

Section: Discussionmentioning

confidence: 99%

Hypertrophic Pachymeningitis and Tracheobronchial Stenosis in IgG4-related Disease: Case Presentation and Literature Review

et al. 2012

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Immunoglobulin G4 (IgG4)-related disease is a distinctive mass-forming disorder with frequent systemic involvement, most commonly in the pancreas, salivary glands and lacrimal glands. A few cases of dural involvement and one case of central airway stenosis have also been described. We report here a rare case of IgG4-related disease with intracranial hypertrophic pachymeningitis and irregular tracheobronchial stenosis. We review four previously reported cases of IgG4-related pachymeningitis. We currently lack international standards for the diagnosis of extrapancreatic IgG4-related disease. Based on the findings of the present case and those reported previously, we discuss the distinctive features of IgG4-related pachymeningitis.

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Recurrent Pleural and Pericardium Effusions in a White Woman With IgG4-related Syndrome

Cited by 29 publications

References 5 publications

IgG4-related Pleuritis with Chylothorax

IgG4-related Pleuritis with Chylothorax

Elevated Serum IgG4 Complicated by Pericardial Involvement with a Patchy <sup>18</sup>F-FDG Uptake in PET/CT: Atypical Presentation of IgG4-related Disease

Hypertrophic Pachymeningitis and Tracheobronchial Stenosis in IgG4-related Disease: Case Presentation and Literature Review

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