Juhong Shi

Pulmonary Manifestations of Sjögren’s Syndrome

Background: Primary Sjögren’s syndrome (PSS) is associated with various histological patterns of interstitial lung disease. Although chest images and lung function studies showed that lung involvement predominantly occurs in small airways, pathological findings were not consistent with the results of high-resolution CT (HRCT) and lung function tests. Objectives: To investigate the pathological characteristics of PSS-associated interstitial lung disease (PSS-ILD) and their relationship with HRCT lung function tests. Methods: Fourteen patients diagnosed as PSS who underwent surgical lung biopsy in Peking Union Medical College Hospital from October 2000 to October 2006 were reviewed. Histopathologic findings, radiologic findings and lung function tests were analyzed. Results:The study included 13 women. The median age was 46 years. Most patients presented with dyspnea and cough. CT scans revealed bilateral ground-glass, consolidative, reticular and nodular opacities and cyst lesions. The histological patterns included nonspecific interstitial pneumonia (NSIP) cellular pattern associated with organizing pneumonia (OP), NSIP mixed pattern associated with OP, noncaseating granulomas, chronic bronchiolitis, follicular bronchiolitis, constrictive bronchiolitis, lymphocytic interstitial pneumonia associated with follicular bronchiolitis, NSIP mixed pattern associated with follicular bronchiolitis, NSIP mixed pattern coexisting with chronic bronchiolitis, OP associated with chronic bronchiolitis, and noncaseating granulomas coexisting with OP. Treatment included prednisone and cyclophosphamide. During the follow-up period (median 38 months), most patients improved or remained stable. The patient with constrictive bronchiolitis died from progression of primary disease. Conclusions: The histopathologic patterns of PSS-ILD included lung interstitial involvement and small airway involvement or both. Corticosteroid therapy combined with cyclophosphamide was administered with a favorable response in the majority of patients.

Clinical Features and Prognosis in 21 Patients with Extrinsic Allergic Alveolitis

OBJECTIVE To assess the spectrum of causes, clinical features, differences between disease phases, and prognosis of extrinsic allergic alveolitis (EAA). METHODS Patients with EAA diagnosed at Peking Union Medical College Hospital from August 1983 to May 2007 were analyzed retrospectively. Their medical records were examined to gather clinical, laboratorial, radiological, and histopathological data. Patients were divided to three phases (acute, subacute, and chronic) according to clinical presentations. Follow-up data regarding treatment response, subsequent radiological and pulmonary function studies, and clinical outcomes were collected. RESULTS A total of 21 cases were enrolled. Among them, 11 were subacute, 10 were chronic. The most common exposure was pet birds (6 cases, 28.6%). The primary abnormality of pulmonary function was restriction and/or reduction in diffusing capacity (12 cases, 63.2%). The most common findings on high-resolution computed tomography (HRCT) were ground-glass opacities (13 cases, 68.4%) and centrilobular nodules (8 cases, 42.1%). Airway obstruction in pulmonary function test, emphysema, lung cysts, and fibrosis on HRCT were more frequently seen in chronic than in subacute patients, though the differences were not statistically significant. Bronchoalveolar lavage fluid (BALF) showed lymphocytosis. The total cell count and the percentage of neutrophils were significantly higher in subacute than in chronic patients (P<0.05). Nonnecrotizing granulomas were seen in 8 (47.1%) cases. Improvement or normalization in symptoms, radiography, and pulmonary function test after treatment were seen in all 18 patients with available follow-up data. Five patients recurred. CONCLUSIONS The characteristic abnormalities of pulmonary function, findings on HRCT, and pathology are essential for all phases of EAA, and the atypical manifestations such as obstruction and fibrosis can also be present frequently, particularly in chronic cases. Differential cell counts of BALF are related to the phase of the disease. The treatment response and prognosis of EAA are good.

Good's Syndrome Patients Hospitalized for Infections: A Single-Center Retrospective Study.

AbstractGoods syndrome (GS) is a rare combination of thymoma and hypogammaglobulinemia, resulting in immunodeficiency. Patients with GS are highly susceptible to bacterial infection, particularly encapsulated bacterial infection in upper and lower respiratory tracts. Goods syndrome patients with moderate-to- severe infection are often hospitalized. Clinical features of GS patients remain to be characterized.Patients with the discharge diagnosis of GS and simultaneous infection from Peking Union Medical College Hospital between January 2001 and July 2015 were retrospectively analyzed.Among 14 hospitalized GS patients, 12 of them were admitted for severe infections. Mean patient age was 56.7 + 10.1 years. Average concentrations of serum IgG, IgA, and IgM were 2.3 + 1.9 g/L, 0.28 + 0.28 g/L, and 0.06 + 0.07 g/L, respectively. Respiratory and intestinal tracts were the most common sites for infection, which occurred in 7 and 4 patients, respectively. Pathogens identified in 10 patients included cytomegalovirus in 5 patients, Pneumocystis jirovecii, Clostridium difficile in 2 patients, Klebsiella pneumonia in 2 patients, and Streptococcus pneumonia and Hemophilus influenza in 1 patient. Ten patients were treated with antibiotics and immunoglobulin replacement. Only 1 patient who was on immunosuppressant therapy died from P. jirovecii pneumonia.Infection was the most frequent cause for hospitalization of GS patients. Both respiratory and intestinal tracts were the most common sites of infection. Cytomegalovirus and P. jirovecii represented 2 common opportunistic pathogens isolated from hospitalized GS patients with infections.

Pathological and High Resolution CT Findings in Churg-Strauss Syndrome

OBJECTIVE To investigate the Churg-Strauss syndrome (CSS) associated lung involvement, concentrating on clinical characteristics, pathological findings of lung involvements, response to treatment, and prognosis. METHODS We retrospectively analyzed the characters of the clinical manifestations, thin-section CT and pathological findings of CSS. The study involved 16 patients. Clinical data were obtained by chart review. All patients underwent transbronchial lung biopsy (TBLB). Six of them underwent surgical lung biopsy as well. RESULTS The patients included 7 men and 9 women, aged from 14 to 61 years (median, 47.5 years). Extrathoracic organs involved included nervous system (7/16) and skin (5/16). Respiratory symptoms included cough (12/16), exertional dyspnea (11/16), hemoptysis (4/16), and chest pain (3/16). CT findings included bilateral ground-glass opacities (12/16), bilateral patchy opacities (12/16), and centrilobular nodules (6/16). The pathological findings of TBLB demonstrated increased eosinophils (3/16), vasculitis (3/16), and interstitial pneumonia (16/16). The pathological findings of surgical lung biopsy of 6 cases showed necrotizing vasculitis in 4 cases, capillaries in 5, eosinophilic pneumonia in 3, granulomas in 2, and airway abnormalities in 3. All patients improved in symptoms after therapy during the study period (range, 3 to 51 months; median, 15 months). CONCLUSIONS Asthma may be present in CSS patient when there is bronchial involvement. Ground-glass opacities and consolidation seen on high-resolution CT reflect the presence of eosinophilic pneumonia, vasculitis, and pulmonary alveolar hemorrhage. TBLB has significant limitations for the diagnosis of CSS. Early diagnosis and therapy can result in satisfactory prognosis.

ABO blood types associated with the risk of venous thromboembolism in Han Chinese people: A hospital-based study of 200,000 patients

ABO blood types are putatively associated with the risk of venous thromboembolism (VTE), but it is not proved in Chinese people. A large population of Han Chinese patients discharged from Peking Union Medical College Hospital between January 2010 and June 2016 were retrospectively analyzed in a case-control study. A total of 1412 VTE patients were identified from 200,660 discharged Han Chinese patients, including 600 patients with deep vein thrombosis (DVT), 441 patients with pulmonary embolism, and 371 patients with both DVT and pulmonary embolism. The prevalence of non-O blood type was weakly but statistically higher in VTE patients compared with 199,248 non-VTE patients, with an odds ratio (OR) of 1.362 (95% confidence interval [CI], 1.205–1.540). Subgroup analysis showed that the OR for non-O blood type was still increased. It was greater in pre-hospital VTE (OR = 1.464) than that in hospital-acquired VTE (OR = 1.224), and greater in unprovoked VTE (OR = 1.859) than that in provoked VTE (OR = 1.227). The OR for non-O blood type decreased with age in subgroup analysis. These results suggest a weak but statistically significant correlation between non-O blood type and risk of VTE in Han Chinese people.

Clinical and diagnostic features of angiosarcoma with pulmonary metastases: A retrospective observational study

Abstract Angiosarcoma with pulmonary metastasis is a rare, fatal disease that often presents with multiple pulmonary nodules and diffuse alveolar hemorrhage. We herein review the detailed clinical characteristics of pulmonary metastatic angiosarcoma and determine a reasonable diagnostic strategy. The medical records of 11 patients with pulmonary angiosarcoma were reviewed. The mean age of the patients was 45.7 years (range, 30–71 years). All patients were male. The most common symptom was hemoptysis (8/11). Common initial misdiagnoses were tuberculosis (5/11), vasculitis (2/11), nontuberculous infectious disease (1/11), and constrictive pericarditis (1/11). Chest computed tomography (CT) of patients with hemoptysis showed bilateral, randomly distributed, variably shaped, and differently sized nodules, as well as ground-glass opacities (GGO) (8/11). The right heart was the most common primary tumor site (8/11), but the sensitivity of echocardiography was limited; CT angiography and cardiac magnetic resonance imaging (MRI) revealed more atrial masses. CT-guided needle biopsy was difficult to perform in most patients because of the small size of the nodules. The diagnosis was made by surgical biopsy of either the lung (3/9) or heart (6/9). The median overall survival of patients who underwent lung biopsy and those who underwent cardiac/pericardiac biopsy was 4.1 and 1.4 months, respectively (P = .098). The median overall survival of the 9 available patients was 5.0 months (95% confidence interval, 0.500–8.544). Angiosarcoma with pulmonary metastases should be considered in patients with hemoptysis and concurrent GGO and nodules on their chest CT scan. Careful cardiologic monitoring is necessary for these patients, even without any cardiac symptoms or signs, and enhanced cardiac MRI is the first recommendation. Surgical biopsy is reliable for histological diagnosis, but the safety of the lung biopsy should be carefully assessed. When primary cardiac tumors are identified, heart biopsy should be preferentially considered.

Intravascular Large B Cell Lymphoma as a Rare Cause of Reversed Halo Sign: A Case Report.

AbstractIntravascular large B cell lymphoma (IVLBCL) is a rare type of extranodal diffused large B-cell lymphoma. IVLBCL with primary lung lesion is very rare and it is very difficult to diagnose. Radiographic findings of pulmonary IVLBCL are nonspecific and resembling interstitial lung diseases. Reversed halo sign (RHS) was initially reported in patients diagnosed with cryptogenic organizing pneumonia and then described in a variety of diseases with inflammatory, infectious, autoimmune, and malignant causes. This is the first case of IVLBCL that has presented with RHSs on CT scan.A 59-year-old Chinese man presented with a 4-month history of a nonproductive cough and a weight loss of 5 kg. Physical examination was unremarkable. High-resolution computed tomography scan of the chest showed bilateral patchy ground glass opacities (GGOs) and RHSs. Laboratory tests were unremarkable except elevated serum lactate dehydrogenase (LDH). Surgical lung biopsy was performed. Light microscopic examination of the specimen disclosed diffuse alveolar septal widening caused by neoplastic lymphocytes, which were positive for CD20 and infiltrated in the alveolar capillaries. The patient was diagnosed with IVLBCL and underwent chemotherapy and autologous blood stem cell transplantation. The patient is still alive 5 years after diagnosis.IVLBCL is a rare cause of RHS and should be considered in differential diagnosis of RHS. An increased serum LDH concentration is another important clue.

Organizing pneumonia components in non-specific interstitial pneumonia (NSIP): a clinicopathological study of 33 NSIP cases

To review the clinical, radiological and pathological features of non‐specific interstitial pneumonia (NSIP), mainly to characterize organizing pneumonia (OP) components in NSIP.

CD4+ and CD8+ T lymphocytes in lung tissue of NSIP: Correlation with T lymphocytes in BALF

BACKGROUND Nonspecific interstitial pneumonia (NSIP) is characterized by the interstitial infiltration T lymphocytes (TLs). Bronchoalveolar lavage fluid (BALF) has been used to analyze the inflammatory cells infiltrating in lung. The controversy about whether the BALF cellular profile reflects T lymphocytes in lung tissue still persists. Some studies found a positive correlation of cell composition between BALF and lung tissue, but others gave opposite conclusion. OBJECTIVE To investigate CD4+ and CD8+ T lymphocytes distribution in lung tissue of NSIP and the relationship with T lymphocytes in bronchoalveolar lavage. METHODS Thirty-seven patients diagnosed as NSIP were included. The pathological and BALF date were reviewed. The characteristics of TLs infiltration in different lung regions were investigated. RESULTS The study included 28 women. The median age was 48 years. In lung tissue, CD4+ and CD8+ lymphocytes (counts/0.1mm2) were separately accounted in lymphoid follicle region (156.51 ± 90.70 vs 85.30 ± 43.75), small blood vessel region (66.58 ± 31.99 vs 58.43 ± 30.24), interstitial region (37.60 ± 19.40 vs 47.12 ± 33.42) and small airway region (26.59 ± 17.04 vs 40.18 ± 34.02). CD4+/CD8+ ratios in lymphoid follicle and small vessel > 1, in interstitium and small airway <1. The number of CD8+ lymphocytes in BALF was correlated with CD8+ lymphocytes around small airway (r = 0.360, p = 0.029) and in interstitial region (r = 0.451, p = 0.005). CD4+/CD8+ ratio in BALF was correlated with that in small airway region (r = 0.437, p = 0.007) and interstitial region (r = 0.468, p = 0.003). CONCLUSIONS In NSIP, T lymphocytes were distributed in different regions of lung tissue. The CD8+ T lymphocytes and CD4+/CD8+ ratio in BALF reflect those in interstitium regions and around small airway of the lung.

Pulmonary hypertension associated with combined fibrosing mediastinitis and bronchial anthracofibrosis: A retrospective analysis in a single Chinese hospital

Both fibrosing mediastinitis (FM) and bronchial anthracofibrosis (BAF) are unique diseases. The combined appearance of FM and BAF is extremely rare.