A case of eosinophilic bronchiolitis complicated with eosinophilic sinusitis]

A 42-year-old man was admitted to our hospital, complaining of dyspnea and cough for six months. Chest CT demonstrated thickening of the bronchial walls and some centrilobular nodules. From the laboratory data and the clinical course, he was first suspected to have bronchial asthma. However, it was necessary to rule out bronchiolitis due to other causes, because he had no previous asthma history and also because of the CT findings. We performed surgical lung biopsy to make a definite diagnosis. The pathological findings revealed eosinophilic bronchiolitis. While dyspnea and the eosinophilic nasal polyp improved by treatment with oral steroid therapy, the eosionophilic sinusitis and bronchiolitis relapsed after steroid tapering. Improvement and relapse of nasal and lower airway symptoms were synchronously observed. Eosinophilic bronchiolitis and eosionophilic sinusitis in this case may be considered to be a category of airway eosinophilic inflammation.     

Clinical characteristics of Japanese patients with eosinophilic esophagitis and eosinophilic gastroenteritis

Background

The clinical characteristics of Japanese patients with eosinophilic esophagitis (EoE) and eosinophilic gastroenteritis (EGE) have not been fully clarified. For understanding the pathogenesis as well as providing support for accurate diagnosis, precise information regarding clinical characteristics of these diseases is important.

Methods

A questionnaire-based survey of EoE and EGE was conducted in 1,078 teaching hospitals. Clinical data of patients with confirmed EoE or EGE diagnosed from 2004 to 2009 were collected.

Result

Clinical data from 26 patients with EoE and 144 patients with EGE were collected. The mean ages of patients in both groups were in the 40s. Those with EoE frequently complained of dysphagia and heartburn, and had characteristic endoscopic features such as longitudinal furrows and multiple concentric rings in the esophagus, while only 34 % had peripheral eosinophilia. Patients with EGE frequently complained of abdominal pain and diarrhea, and approximately 80 % of them have peripheral eosinophilia. They did not have characteristic endoscopic features helpful for diagnosis. Computed tomography (CT) findings and the presence of peripheral eosinophilia were diagnostic for EGE. EGE patients with a small intestinal involvement showed the highest peripheral eosinophil counts. Glucocorticoid administration was the most widely used treatment for these diseases and its effect was favorable for at least induction of remission.

Conclusion

EGE is more prevalent than EoE in Japan. Patients with EGE have abdominal pain and diarrhea, high peripheral eosinophil counts, and gastrointestinal wall thickening identifiable by CT findings, while EoE is characterized by dysphagia and characteristic endoscopic features.     

A diagnostic score for eosinophilic granulomatosis with polyangiitis among eosinophilic disorders

BackgroundEosinophilic granulomatosis with polyangiitis (EGPA) is a form of systemic vasculitis with eosinophilic inflammation. However, existing classification criteria are all designed to classify EGPA among vasculitis and there is no established method distinguishing EGPA from other eosinophilic disorders. The aim of the present study was to propose a scoring system to differentiate EGPA among eosinophilic disorders.MethodsNon-supervised hierarchical clustering using Ward's method and principal component analysis (PCA) were performed for 19 clinical parameters of 58 patients with eosinophilia-related diseases at a tertiary university hospital. The newly proposed scoring system was externally validated in 40 patients at another tertiary institution.ResultsTwo distinct clusters were identified, and clinical features including peripheral neuropathy, asthma, skin involvement, lung involvement, rheumatoid factor (RF) positivity, myeloperoxidase (MPO)–anti-neutrophil cytoplasmic antibody (ANCA) positivity, IgE elevation, C-reactive protein (CRP) elevation, and vasculitis pathological findings were predominantly observed in one of these clusters (p < 0.05). Ten features defining the cluster with a high rate of vasculitis were weighted by PCA to create the E-CASE (EGPA classification among systemic eosinophilia) scoring system, on a 16-point scale. Based on the distribution of scores in the primary cohort, we defined an E-CASE score ≥12 as positive, ≤ 8 as negative, and 9–11 as undeterminable. The sensitivity and specificity of the E-CASE score in the validation cohort were 93.3% and 100%, respectively.ConclusionsWe developed and verified a novel scoring system for differentiating EGPA from other types of eosinophilic disorders.     

Chronic bronchiolitis with associated eosinophilic lung disease (eosinophilic bronchiolitis).

We encountered an adult patient with dyspnea, eosinophilia and bronchiolitis. He was diagnosed as having diffuse panbronchiolitis, and was treated with erythromycin for 3 years, but his symptoms had gradually worsened. Bronchoalveolar lavage showed marked eosinophilia, and video-assisted thoracoscopic lung biopsy revealed chronic bronchiolitis with associated eosinophilic lung disease (eosinophilic bronchiolitis). To our knowledge, no case of eosinophilic bronchiolitis has been reported in the literature.     

Update on eosinophilic granulomatosis with polyangiitis

Eosinophilic granulomatosis with polyangiitis (EGPA) (formerly Churg-Strauss syndrome) is a rare form of anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis characterized by eosinophil-rich granulomatous inflammation and small to medium-size vessel vasculitis associated with bronchial asthma and eosinophilia. Its rarity and unique features such as eosinophilic inflammation have delayed progress of research regarding EGPA for several years, compared to other forms of ANCA-associated vasculitis. However, recently, attention to EGPA as a research subject has been gradually increasing. To resolve problems in existing criteria for EGPA, new classification criteria for EGPA generated by a large international cohort will be launched and is being expected to accelerate future studies. Pathogenesis and roles of ANCA in EGPA are still largely unknown; however, it has been reported that glomerulonephritis is more frequent in ANCA-positive patients than in ANCA-negative patients, while heart failure is more frequent in ANCA-negative patients than in ANCA-positive patients. In addition, a recent genome-wide association study has suggested the presence of two genetically distinct subgroups of EGPA, which correspond to ANCA-positive and -negative subgroups. Although responses to glucocorticoids in EGPA are generally good, patients with EGPA often experience a relapse. Currently, there is no standard therapy for EGPA based on accumulation of clinical trial results. Recently, clinical benefits of mepolizumab for EGPA were proved by a randomized controlled trial and mepolizumab was approved for EGPA. In addition, various new drugs are under evaluation. To find optimal use of these drugs and to resolve unmet needs, such as relapse prevention, will be needed in future.     

Eosinophilic pneumonia with eosinophilic gastroenteritis.

A 48-year-old man was admitted to our hospital with cough, fever and dysphagia. He had a past history of bronchial asthma and surgery for nasal polyp. Chest radiograph and computed tomography showed atelectasis in the right lower field and infiltrative shadow in the left lower field and overall thickening of the esophageal wall. Transbronchial lung biopsy (TBLB) specimens revealed infiltration of eosinophils and lymphocytes under the bronchial mucosa. Gastrointestinal tract biopsy specimens showed submucosal infiltration of eosinophils. These findings led to a definite diagnosis of eosinophilic pneumonia associated with eosinophilic gastroenteritis, a disease which has been rarely reported.     

Acute nonlymphocytic leukemia with eosinophilic differentiation

Four cases of de novo acute nonlymphocytic leukemia (ANLL) with early eosinophilic differentiation are described. The clinical course did not differ from that of the usual forms of ANLL. Morphologic and cytochemical features that can support this diagnosis are discussed. Particularly, the cyanide-resistant peroxidase stain appeared to be a specific marker of eosinophilic differentiation. Acute eosinophilic leukemia is a distinct entity, and this unusual subtype of ANLL can be set apart from other forms of ANLL characterized by hypereosinophilia.     

Idiopathic eosinophilic myositis with preexisting fibromyalgia

Idiopathic eosinophilic myositis is a rare entity. Only 6 other cases have been reported in the literature. We report a patient with degenerative joint disease and fibromyalgia who subsequently developed eosinophilic myositis over a tender trigger area.     

Pseudoeosinophilia, eosinophilic endocarditis and eosinophilic leukemia

Three patients with eosinophilia of diverse etiology are described. All three had cardiopulmonary symptoms and hepatosplenomegaly in addition to the eosinophilia. One patient was found to have reactive eosinophilia and lesions in the heart typical of Loeffler's endocarditis. The second patient had eosinophilic leukemia and Loeffler's endocarditis. The third patient had false eosinophilia confirmed by cytochemical and electron microscopic studies. Leukocyte alkaline phosphatase, chromosomal study of marrow cells and response to chemotherapy were not helpful in differentiating reactive eosinophilia from neoplastic eosinophilia. Cytologic evaluation of the blood cells may be helpful, but its actual value needs further study for confirmation. In dealing with eosinophilia of unknown etiology, false eosinophilia is a new possibility that should be considered.     

Deposits of eosinophil granule proteins in eosinophilic cholecystitis and eosinophilic colitis associated with hypereosinophilic syndrome

Summary A case of hypereosinophilic syndrome with eosinophilic colitis, eosinophilic cholecystitis, and increased serum levels of interleukin-5 (IL-5) and soluble interleukin-2 receptor (sIL-2R) is reported. Immunohistochemical studies of cholecystectomy and colon biopsy specimens with monoclonal antibodies, which are specific for activated eosinophils, secreted eosinophil cationic protein (ECP) and for major basic protein (MBP), demonstrated the presence of numerous activated eosinophils, secretion of ECP, and deposition of MBP in areas of tissue damage. These findings suggest that in eosinophilic cholecystitis and eosinophilic colitis, activated eosinophils infiltrate and degranulate in each tissue, releasing eosinophil granule proteins that produce tissue damage.     

Hypercalcemia in a patient with eosinophilic granuloma

Persistent hypercalcemia subsequently developed in a 46-year-old black woman with biopsy-proved eosinophilic granuloma. Evaluation of this patient excluded or made highly unlikely any of the known causes of hypercalcemia. As with several other granulomatous diseases, eosinophilic granuloma is likely an occasional cause of hypercalcemia.     

Chronic eosinophilic pneumonia with pleural effusion.

The case history of a 77-year-old lady with chronic eosinophilic pneumonia is presented. The diagnosis was difficult due to the simultaneous presence of a pleural effusion and congestive heart failure. Radiological findings and treatment are discussed.     

Acid hypersensitivity in patients with eosinophilic oesophagitis

Abstract

Objective. Painful symptoms are prevalent in patients with eosinophilic oesophagitis but experimental data are sparse. The aim of this study was to compare the pain response to experimental oesophageal stimulation in 14 patients with eosinophilic oesophagitis and 15 healthy volunteers. Material and methods. A multimodal probe was placed in the oesophagus. The participants were subjected to mechanical, thermal and electrical pain stimuli followed by perfusion with 0.1 M HCl. Pain scores, referred pain areas and evoked brain potentials to electrical stimulation of the oesophagus were recorded. Results. Patients tolerated significantly less acid perfused in the oesophagus (median 123 versus 200 ml; P = 0.02) and felt the burning sensation evoked by the acid earlier (median 2.0 versus 5.0 min; P = 0.01). Eight patients had coexisting gastro-oesophageal reflux disease. Six patients had pure eosinophilic oesophagitis, and this group felt the acid earlier than those with concomitant reflux or the healthy volunteers (median 0.8 versus 2.0 and 5.0 min; P = 0.03). There were no differences between patients and controls in the responses to mechanical or thermal stimulation (P >?0.4). Furthermore, no differences were found for the proxies of central nervous system sensitization (response to electrical stimulations, referred pain areas or evoked brain potentials; P >?0.1). Conclusions. Patients with eosinophilic oesophagitis are hypersensitive to acid perfused in the oesophagus, and pathophysiologic findings are likely confined to the peripheral tissue. Reflux from physiological acid may play a role in the symptoms of eosinophilic oesophagitis.