Chinese Expert Consensus on the Diagnosis and Treatment of Inflammatory Myofibroblastic Tumor.

Inflammatory myofibroblastic tumor (IMT) is a rare spindle-cell neoplasm. IMT currently suffers from a paucity of standardized diagnostic and therapeutic guidelines. The Chinese expert consensus committee on the diagnosis and treatment of IMT formed an "Expert consensus on the diagnosis and treatment of inflammatory myofibroblastic tumor".

Transcriptional regulation and small compound targeting of ACE2 in lung epithelial cells.

Angiotensin-converting enzyme 2 (ACE2) is the receptor of COVID-19 pathogen SARS-CoV-2, but the transcription factors (TFs) that regulate the expression of the gene encoding ACE2 (ACE2) have not been systematically dissected. In this study we evaluated TFs that control ACE2 expression, and screened for small molecule compounds that could modulate ACE2 expression to block SARS-CoV-2 from entry into lung epithelial cells. By searching the online datasets we found that 24 TFs might be ACE2 regulators with signal transducer and activator of transcription 3 (Stat3) as the most significant one.

Distinctive Clinical Characteristics and Outcome of ILD-Onset Rheumatoid Arthritis and ACPA-Positive ILD: a Longitudinal Cohort of 282 Cases.

The aim of this study is to investigate the clinical features and outcome of interstitial lung disease (ILD)-onset rheumatoid arthritis (RA) and anti-citrullinated protein antibody (ACPA)-positive ILD-only patients. Arthritis-onset and ILD-onset RA-ILD and ACPA-positive ILD-only patients consecutively admitted to Peking Union Medical College Hospital from January 2008 to December 2017 were enrolled and followed-up. Their demographic, clinical, and laboratory features as well as outcome were collected and analyzed.

Association between clinicopathologic characteristics and BRAF expression in Chinese patients with Langerhans cell histiocytosis.

Background: The identification of V-raf murine sarcoma viral oncogene homolog B1 (BRAF) mutations has been recommended in patients with Langerhans cell histiocytosis (LCH) with difficult diagnosis and failure of first-line treatment. The reported frequencies of BRAF mutations vary in Chinese patients with LCH.

Methods: We conducted a retrospective analysis of LCH patients with a definitive pathological diagnosis who were hospitalized between 2013 and 2017.

Langerhans cell histiocytosis of the thyroid complicated by papillary thyroid carcinoma: A case report and brief literature review.

Rationale: Langerhans cell histiocytosis (LCH) involves mainly the skin and bone and rarely the thyroid. Meanwhile, papillary thyroid carcinoma (PTC) is the most common subtype of thyroid cancer. Both LCH and PTC could make the thyroid enlarged and hypermetabolic.

Gliomatosis peritonei: a series of eight cases and review of the literature.

Background: Gliomatosis peritonei (GP) is a rare condition characterized by mature glial tissue implants widespread in the peritoneum. The GP is often associated with ovarian teratoma. However, little is known about the characteristics and prognosis of GP.

A case report: The efficacy of pirfenidone in a Chinese patient with progressive systemic sclerosis-associated interstitial lung disease: A CARE-compliant article.

Systemic sclerosis (SSc)-associated interstitial lung disease (SSc-ILD) has become the leading SSc-related cause of death. Although various types of immunosuppressive therapy have been attempted for patients with SSc-ILD, no curative or effective treatment strategies for SSc-ILD have been developed. Therefore, management of patients with SSc-ILD remains a challenge.

Types of Organ Involvement in Patients with Immunoglobulin G4-related Disease.

Background: Immunoglobulin G4-related disease (IgG4-RD) is a newly recognized systemic disease that can involve multiple organs and various clinical phenotypes. The purpose of this study was to analyze different types of organ involvement in IgG4-RD patients in China.

Methods: We conducted a prospective cohort study on IgG4-RD patients to analyze the clinical manifestations and rare features of IgG4-RD.

Computed Tomographic and Pathological Features of Primary Pulmonary Sarcomatoid Carcinoma.

Objective: To investigate the computed tomographic (CT) and pathological features of primary pulmonary sarcomatoid carcinoma (PSC).

Methods: The clinical data and CT images of 20 patients with pathologically confirmed PSC were retrospectively analyzed.

Results: Solitary pulmonary mass was identified in 18 patients and multiple pulmonary masses in 2 patients, amounting to 22 masses.

[The clinical-radiologic-pathologic features of imported pulmonary histoplasmosis].

Objective: To describe the clinical features and treatment of imported pulmonary histoplasmosis and therefore to improve the recognition and differential diagnosis of this disease.

Methods: The clinical data of 3 patients with imported pulmonary histoplasmosis in our hospital were collected and analyzed. Literatures published since 1989 were retrieved with 'pulmonary histoplasmosis' from PubMed, China National Knowledge Infrastructure (CNKI), Wanfang Data and VIP data, of which all the literatures about imported pulmonary histoplasmosis were reviewed.

[Surgical lung biopsy for the management of diffuse interstitial lung disease].

Objective: To evaluate the role of surgical lung biopsy (SLB) in the management of diffuse interstitial lung disease (DILD) with no specific diagnosis.

Methods: We conducted a retrospective analysis of 179 cases with DILD of non-specific diagnosis after non-invasive methods and minimally invasive processes, including bronchoscopy and CT-guided lung biopsy, were performed SLB at Peking Union Medical College Hospital between 2006 and 2012. Patient demographics, surgical approach, number and site of biopsies, post-operative complications and postoperative pathological diagnosis and treatment were analyzed.

[The clinicopathological analysis of pulmonary parenchymal involvement of multicentric giant lymph node hyperplasia (Castleman's disease)].

Objective: To observe the clinicopathological features of pulmonary parenchymal involvement of multicentric Castleman's disease(MCD).

Methods: Retrospective analysis was carried out for 6 patients of MCD with pulmonary parenchymal involvement who had been admitted to Peking Union Medical College Hospital from July 2008 to March 2013. Relevant literatures were reviewed.

[Clinical characteristic analysis of 96 cases of hypersensitivity pneumonitis].

Objective: To improve understanding of the clinical characteristics and diagnosis of hypersensitivity pneumonitis (HP).

Methods: We retrospectively analyzed the clinical data, including clinical symptoms, laboratory tests, exposure, pulmonary function tests, chest CT imaging and cytological classification of bronchoalveolar lavage (BAL) of 96 patients with HP from Jan 2001 to Jun 2011 in Peking Union Medical College Hospital. We divided the patients into 2 groups: a pathologically-confirmed group and a clinically-suspected group.

[Study on pathological characteristics of eutopic endometrium in endometriosis].

Objective: To study the pathologic characteristics of eutopic endometrium in patients with endometriosis.

Methods: Pathologic characteristics of eutopic endometrium were studied in 176 patients with endometriosis in Peking Union Medical College Hospital from January 2007 to December 2008 retrospectively.

Results: About 72.

[IgG4-related lung disease: a case report and review of the literature].

Objective: To describe the clinical features of IgG4-related lung disease.

Method: The clinical symptoms, laboratory tests, radiographic patterns, histopathological features and therapeutic management of a patient with IgG4-related lung disease were described and the literatures were reviewed.

Results: A 41-year-old male without significant symptoms was admitted to our department because of diffuse opacities on regular X-ray examination.

[The clinicopathological analysis of 4 cases of IgG4-related nonspecific interstitial pneumonia].

Objectives: To observe the immunohistochemical staining of IgG4 in nonspecific interstitial pneumonia (NSIP) and to study the clinicopathological features of IgG4-related NSIP.

Methods: Retrospective analysis was carried out for 32 patients with NSIP who had been admitted into Peking Union Medical College Hospital from November 2002 to October 2010. The diagnosis of NSIP was established by surgical lung biopsy and all specimens were fixed in neutral formalin and embedded in paraffin.

[The diagnostic value of CT-guided percutaneous needle lung biopsy in diffuse parenchymal lung diseases].

Objective: This study was to evaluate the efficacy and limitation of CT-guided percutaneous cutting needle lung biopsy in the diagnosis of diffuse parenchymal lung diseases (DPLD).

Methods: A total of 481 patients admitted in Peking Union Medical College Hospital from January 2000 to December 2008 underwent CT-guided percutaneous cutting needle lung biopsy. The patients were evaluated by clinical history, physical examination and lung HRCT.

[Clinical and imaging manifestations of pulmonary mucosa-associated lymphoid tissue lymphoma].

Objective: To investigate the clinical and computed tomography (CT) appearances of pulmonary mucosa-associated lymphoid tissue (MALT) lymphoma.

Methods: The CT findings and clinical data of 13 patients with pathologically proven pulmonary MALT lymphoma were retrospectively reviewed.

Results: Among these 13 patients, seven presented no notable abnormalities, six manifested respiratory symptoms including cough, expectoration, and dyspnea; one of these six patients experienced fever.

[Pulmonary lymphomatoid granulomatosis: an immunohistochemical and gene rearrangement study].

Objective: To study the immunophenotype and gene rearrangement pattern of pulmonary lymphomatoid granulomatosis.

Methods: Nine cases of pulmonary lymphomatoid granulomatosis, included 5 cases of open lung biopsy, 3 cases of lobectomy specimen and 1 case of autopsy, were retrospectively analyzed by immunohistochemistry, in-situ hybridization for Epstein-Barr virus-encoded RNA, immunoglobulin and T-cell receptor gene rearrangement studies.

Results: The age of patients ranged from 3 to 59 years.