scholarly journals Eosinophilic bronchiolitis successfully treated with benralizumab

2021 ◽  
Vol 14 (10) ◽  
pp. e246058
Author(s):  
Keishi Sugino ◽  
Hirotaka Ono ◽  
Akira Hebisawa ◽  
Eiyasu Tsuboi
Keyword(s):  
Plasma Cells ◽  
Final Diagnosis ◽  
Japanese Woman ◽  
Wall Thickening ◽  
High Dose ◽  
Immunoglobulin G4 ◽  
Bronchial Wall ◽  
History Of ◽  
Frequent Exacerbation ◽  
Wet Cough

A 53-year-old non-smoking Japanese woman was admitted to our hospital with a 20-year history of wet cough and dyspnoea on exertion. Bronchial asthma (BA) had been diagnosed 20 years earlier. Although she has been treated with high-dose inhaled corticosteroid, she had experienced frequent exacerbation of BA, and short-term oral corticosteroid bursts were occasionally administered. High-resolution CT of the chest revealed diffuse centrilobular nodules with bronchial wall thickening and patchy ground-glass opacities in both lungs. Lung biopsy specimens showed widespread cellular bronchiolitis with follicle formations in the membranous and respiratory bronchioles, accompanied by marked infiltration of plasma cells and eosinophils. In addition, immunohistochemical immunoglobulin G4 (IgG4) staining revealed many IgG4-positive plasma cells, and the ratio of IgG4-positive cells to IgG-positive cells exceeded 40%. The final diagnosis was eosinophilic bronchiolitis with marked IgG4-positive plasma cell infiltration in association with BA. With benralizumab therapy, her clinical condition dramatically improved.

scholarly journals Immunoglobulin G4-related sclerosing cholangitis mimicking cholangiocarcinoma: a case report and literature review

2020 ◽  
Vol 48 (10) ◽  
pp. 030006052095921
Author(s):  
Cheng Xu ◽  
Yongmei Han
Keyword(s):  
Sclerosing Cholangitis ◽  
Plasma Cells ◽  
Elevated Serum ◽  
Clinical Manifestations ◽  
Immunoglobulin G4 ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
History Of ◽  
The Common

Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a novel clinical disease that is characterized by elevated serum IgG4 concentrations and tumefaction or tissue infiltrated by IgG4+ plasma cells. The clinical manifestations of IgG4-RD depend on the type of tissues affected. IgG4-related sclerosing cholangitis is a type of IgG4-RD. We report a patient who initially visited a local hospital with a 5-month history of jaundice. He was found to have a mass in the upper part of the common bile duct that mimicked cholangiocarcinoma. He underwent surgery in our hospital and was later diagnosed with IgG4-related sclerosing cholangitis. We administered prednisolone 40 mg once a day for treatment. Taking into account the possible side effects of moderate-dose hormone therapy, we also administered teprenone, potassium chloride, and calcium carbonate. The patient did not have any recurrence of symptoms or adverse drug reactions during follow-up.

scholarly journals Unique angiographic findings in a patient with myocardial ischemia and immunoglobulin G4-related disease

2017 ◽  
Vol 5 ◽  
pp. 2050313X1772801
Author(s):  
Yusuke Kanzaki ◽  
Takashi Miura ◽  
Naoto Hashizume ◽  
Tatsuya Saigusa ◽  
Soichiro Ebisawa ◽  
...  
Keyword(s):  
Coronary Artery Disease ◽  
Coronary Artery ◽  
Coronary Angiography ◽  
Intravascular Ultrasound ◽  
Plasma Cells ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Multiple Organs ◽  
History Of ◽  
Artery Disease

Immunoglobulin G4-related disease characteristically involves multiple organs including the heart and coronary arteries. Immunoglobulin G4-related coronary artery disease is thought to be due to periarteritis and histopathologically is characterized by marked thickening of the adventitia and periarterial fat with infiltration of immunoglobulin G4-positive plasma cells. Although comprehensive diagnostic criteria require a biopsy for a definite or probable diagnosis of immunoglobulin G4-related disease, obtaining a coronary artery biopsy is difficult and risky. However, imaging findings including coronary angiography and intravascular ultrasound might be useful tools to establish a diagnosis of immunoglobulin G4-related coronary artery disease. We report a case of a 63-year-old man with a history of immunoglobulin G4-related disease who presented with exertional chest pain. We found unique angiographic and intravascular ultrasound features of immunoglobulin G4-related coronary artery disease that distinguished it from those of arteriosclerotic coronary artery disease and suggest that coronary angiography and intravascular ultrasound might be useful tools in the diagnosis of immunoglobulin G4-related coronary artery disease.

scholarly journals Hypertrophic pachymeningoencephalitis associated with temporal giant cell arteritis

2018 ◽  
pp. bcr-2018-225304
Author(s):  
Gesine Boisch ◽  
Sven Duda ◽  
Christian Hartmann ◽  
Heinrich Weßling
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Temporal Arteritis ◽  
Brain Mri ◽  
Neurological Deficits ◽  
High Dose ◽  
Immunoglobulin G4 ◽  
Perifocal Oedema ◽  
History Of ◽  
Dose Corticosteroid

We report the rare case of an 80-year-old male patient with hypertrophic pachymeningoencephalitis that may be associated with temporal arteritis. The patient presented to our neurological department with a 2-week history of latent paresis and ataxia affecting his right hand. He had been diagnosed with temporal arteritis 12 years earlier. Brain MRI showed an enhancement of the left-sided frontoparietal meninges with oedema of the adjacent tissue of the precentral and postcentral cortex. A leptomeningeal biopsy was performed. An autoimmune-mediated immunoglobulin G4-associated hypertrophic pachymeningoencephalitis was diagnosed. The patient received a high-dose corticosteroid therapy and his symptoms gradually improved. Our results suggest that hypertrophic pachymeningoencephalitis may occur as a complication of giant cell arteritis and may cause central neurological deficits by cerebral perifocal oedema.

IgG4-related tubulointerstitial nephritis

2021 ◽  
Vol 14 (11) ◽  
pp. e241942
Author(s):  
Tasnim Momoniat ◽  
Deepa Jacob ◽  
Neelaveni Duhli ◽  
Tom Jorna
Keyword(s):  
Plasma Cells ◽  
Tubulointerstitial Nephritis ◽  
Epigastric Pain ◽  
Kidney Injury ◽  
Total Serum Protein ◽  
Total Serum ◽  
Immunoglobulin G4 ◽  
Igg4 Related Disease ◽  
History Of ◽  
Steroid Sensitive

A 67-year-old man was referred to the renal team following an episode of acute kidney injury on a background of chronic kidney disease. He had a 9-year history of steroid-sensitive arthritis, epigastric pain and isolated submandibular gland enlargement. He was noted to have a raised eosinophil count, total serum protein and total immunoglobulin G4 (IgG4) level as well as a serum hypocomplementaemia. A renal biopsy showed a tubulointerstitial nephritis with lymphoplasmacytic infiltrates, fibrosis and IgG4-positive plasma cells on immunohistochemistry. A diagnosis of IgG4-related disease was made based on clinical presentation and pathology. Renal function improved with glucocorticoids and the patient was successfully transitioned to azathioprine as a steroid-sparing agent.

scholarly journals Effect of Sulfur Dioxide Inhalation on Lung Microbiota in Rat Model

2020 ◽  
pp. 21-26
Author(s):  
Xiaoyu Li ◽  
Qianru Wang ◽  
Meng Wang
Keyword(s):  
Sulfur Dioxide ◽  
High Throughput Sequencing ◽  
Male Rats ◽  
Control Group ◽  
Wall Thickening ◽  
High Dose ◽  
Exposure Group ◽  
Bronchial Wall ◽  
And Control ◽  
Pathological Structure

Purpose: To investigate the effect of sulfur dioxide on the lung microbiota of healthy rats. Methods Fifteen male rats were randomly divided into high dose and low dose exposure group and control group. After 7 days of SO2 exposure, the lung tissues were obtained and the lung microbiota was identified by Illumina high-throughput sequencing. Results The microbial community of lung microbiota was significantly alternated in the exposure group and the dominant phylum changed from Firmicutes to Proteobacteria. In addition, the SO2 exposure caused the bronchial wall thickening and a large number of inflammatory cell infiltration in the lungs of rats in exposure groups. Conclusions The results suggest that SO2 can significantly alter the lung microbiota and pathological structure of the lungs.

scholarly journals COVID-19 versus H1N1: challenges in radiological diagnosis—comparative study on 130 patients using chest HRCT

2021 ◽  
Vol 52 (1) ◽  
Author(s):  
Ahmed Samir ◽  
Nagy N. N. Naguib ◽  
Abdelaziz Elnekeidy ◽  
Ayman Ibrahim Baess ◽  
Amal Shawky
Keyword(s):  
Influenza Season ◽  
Influenza Viruses ◽  
The Other ◽  
Wall Thickening ◽  
Significant Relation ◽  
Air Trapping ◽  
Bronchial Wall ◽  
History Of ◽  
Second Wave ◽  
Traction Bronchiectasis

Abstract Background During the current second wave of COVID-19, the radiologists are expected to face great challenges in differentiation between COVID-19 and other virulent influenza viruses, mainly H1N1. Accordingly, this study was performed in order to find any differentiating CT criteria that would help during the expected clinical overlap during the current Influenza season. Results This study was retrospectively conducted during the period from June till November 2020, on acute symptomatic 130 patients with no history of previous pulmonary diseases; 65 patients had positive PCR for COVID-19 including 50 mild patients and 15 critical or severe patients; meanwhile, the other 65 patients had positive PCR for H1N1 including 50 mild patients and 15 critical or severe patients. They included 74 males and 56 females (56.9%:43.1%). Their age ranged 14–90 years (mean age 38.9 ± 20.3 SD). HRCT findings were analyzed by four expert consultant radiologists in consensus. All patients with COVID-19 showed parenchymal or alveolar HRCT findings; only one of them had associated airway involvement. Among the 65 patients with H1N1; 56 patients (86.2%) had parenchymal or alveolar HRCT findings while six patients (9.2%) presented only by HRCT signs of airway involvement and three patients (4.6%) had mixed parenchymal and airway involvement. Regarding HRCT findings of airway involvement (namely tree in bud nodules, air trapping, bronchial wall thickening, traction bronchiectasis, and mucous plugging), all showed significant p value (ranging from 0.008 to 0.04). On the other hand, HRCT findings of parenchymal or alveolar involvement (mainly ground glass opacities) showed no significant relation. Conclusion HRCT can help in differentiation between non-severe COVID-19 and H1N1 based on signs of airway involvement.

scholarly journals Chronic massive pericardial effusion: a case report and literature review

2020 ◽  
Vol 48 (11) ◽  
pp. 030006052097309
Author(s):  
Ying-shuo Huang ◽  
Jian-xiong Zhang ◽  
Ying Sun
Keyword(s):  
Pericardial Effusion ◽  
Cardiac Tamponade ◽  
Severe Heart Failure ◽  
Final Diagnosis ◽  
High Dose ◽  
Indwelling Catheter ◽  
Large Pericardial Effusion ◽  
History Of ◽  
Lymph Node Tuberculosis

Chronic massive pericardial effusion without cardiac tamponade is relatively rare. Nearly half of all patients with chronic large pericardial effusion are asymptomatic. We report a case of a 77-year-old man who presented with an asymptomatic chronic massive pericardial effusion, with no evidence of cardiac tamponade or pericardial constriction during a 10-year follow-up. The patient had a complex history of lymph node tuberculosis, hypertension, hypothyroidism, and polycythemia vera, as well as high-dose 31P radiation exposure 45 years ago. There was no evidence of tuberculosis infection, hypothyroidism, malignant tumor, severe heart failure, uremia, trauma, severe bacterial or fungal infection, chronic myeloid leukemia, or bone marrow fibrosis after admission. The patient underwent pericardiocentesis twice. The pericardial effusion comprised exudate fluid with a high proportion of monocytes. The patient refused indwelling catheter drainage or pericardiectomy. The likely final diagnosis was recurrent chronic large idiopathic pericardial effusion.

scholarly journals Pulmonary changes on HRCT scans in nonsmoking females with COPD due to wood smoke exposure

2013 ◽  
Vol 39 (2) ◽  
pp. 155-163 ◽  
Author(s):  
Maria Auxiliadora Carmo Moreira ◽  
Maria Alves Barbosa ◽  
Maria Conceição de Castro Antonelli Monteiro de Queiroz ◽  
Kim Ir Sen Santos Teixeira ◽  
Pedro Paulo Teixeira e Silva Torres ◽  
...  
Keyword(s):  
Demographic Data ◽  
Positive Association ◽  
Smoke Exposure ◽  
Wood Smoke ◽  
Control Group ◽  
Wall Thickening ◽  
Bronchial Wall ◽  
History Of ◽  
Pulmonary Changes ◽  
And Control

OBJECTIVE: To identify and characterize alterations seen on HRCT scans in nonsmoking females with COPD due to wood smoke exposure. METHODS: We evaluated 42 nonsmoking females diagnosed with wood smoke-related COPD and 31 nonsmoking controls with no history of wood smoke exposure or pulmonary disease. The participants completed a questionnaire regarding demographic data, symptoms, and environmental exposure. All of the participants underwent spirometry and HRCT of the chest. The COPD and control groups were adjusted for age (23 patients each). RESULTS: Most of the patients in the study group were diagnosed with mild to moderate COPD (83.3%). The most common findings on HRCT scans in the COPD group were bronchial wall thickening, bronchiectasis, mosaic perfusion pattern, parenchymal bands, tree-in-bud pattern, and laminar atelectasis (p < 0.001 vs. the control group for all). The alterations were generally mild and not extensive. There was a positive association between bronchial wall thickening and hour-years of wood smoke exposure. Centrilobular emphysema was uncommon, and its occurrence did not differ between the groups (p = 0.232). CONCLUSIONS: Wood smoke exposure causes predominantly bronchial changes, which can be detected by HRCT, even in patients with mild COPD.

scholarly journals Primary plasma cell leukaemia in a 39-year-old man

2020 ◽  
Vol 13 (7) ◽  
pp. e235543
Author(s):  
Ganesh Kasinathan
Keyword(s):  
Plasma Cell ◽  
Plasma Cells ◽  
Autologous Stem Cell Transplant ◽  
Blood Film ◽  
Cell Leukaemia ◽  
High Dose ◽  
Cell Transplant ◽  
Plasma Cell Leukaemia ◽  
History Of ◽  
High Dose Melphalan

Plasma cell leukaemia (PCL) is an aggressive haematological malignancy which is classified into primary (pPCL) and secondary PCL. A 39-year-old Indian man presented to the Department of Hematology with a 2-week history of fever and lethargy. Clinically, he was pale and febrile. Haemogram revealed bicytopenia with leucocytosis. The peripheral blood film portrayed rouleax formation with 45% of circulating plasma cells. Serum protein electrophoresis and immunofixation revealed IgG lambda paraproteinaemia of 48 g/L. Bone marrow aspirate, flow cytometry and trephine were consistent with IgG lambda pPCL. He was treated with six cycles of bortezomib, thalidomide and dexamethasone combination chemotherapy followed by high-dose melphalan conditioning and autologous stem cell transplant. Currently, he is in complete remission for the past 18 months and is on oral lenalidomide maintenance therapy. Prognosis is often dismal in pPCL with the median overall survival below 1 year if treatment is delayed.

scholarly journals Immunoglobulin G4 hypophysitis in a 63-year-old woman with no autoimmune history: a case report

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Zachary C. Gersey ◽  
Kenan R. Rajjoub ◽  
Thomas M. Pearce ◽  
Scott A. Segel ◽  
Paul A. Gardner ◽  
...  
Keyword(s):  
Autoimmune Disease ◽  
Response To Treatment ◽  
White Female ◽  
High Dose ◽  
Demographic Differences ◽  
Immunoglobulin G4 ◽  
Case Presentation ◽  
Cranial Nerve Palsies ◽  
History Of ◽  
Pituitary Lesion

Abstract Background Immunoglobulin-G4-related hypophysitis is a rare inflammatory disease that can present as a tumefactive pituitary lesion mimicking hypophyseal neoplasms such as pituitary adenoma or craniopharyngioma. The literature on this entity is sparse, with fewer than 100 cases reported across 19 publications; a recent review found only 24 cases published from 2007 to 2018. Previous reports have described demographic differences, with immunoglobulin-G4-related hypophysitis in females tending to present in the second and third decades in association with other autoimmune disease, while males tend to present in the fifth and sixth decades of life without an autoimmune history. Case presentation In contrast to the reported demographic trends, here we describe a unique case of immunoglobulin-G4-related hypophysitis in a 63-year-old white female with no history of autoimmune disease who presented with a rapidly enlarging sellar and hypothalamic mass causing headaches and cranial nerve palsies, prompting biopsy for diagnosis. The patient experienced rapid response to treatment with high-dose steroids and rituximab. Conclusion The case contributes to the growing clinicopathologic description of immunoglobulin-G4-related hypophysitis and illustrates that this diagnosis should be a consideration even outside the conventional demographic setting.

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