Meng Fq

Clinical and Radiological Profile of Acute Fibrinous and Organizing Pneumonia: A Retrospective Study

Background: Acute fibrinous and organizing pneumonia (AFOP) is a unique pathological entity with intra-alveolar fibrin in the form of “fibrin balls” and organizing pneumonia. It was divided into rare idiopathic interstitial pneumonia according to the classification notified by American Thoracic Society/European Respiratory Society in 2013. As a rare pathological entity, it is still not well known and recognized by clinicians. We reviewed the clinical features of 20 patients with AFOP diagnosed in a teaching hospital. Methods: The medical records of 20 patients with biopsy-proven diagnosis of AFOP were retrospectively reviewed. The patients’ symptoms, duration of the disease, comorbidities, clinical laboratory data, pulmonary function testing, radiographic studies, and the response to treatment were extracted and analyzed. Results: Fever was the most common symptom and was manifested in 90% of AFOP patients. For clinical laboratory findings, systematic inflammatory indicators, including C-reactive protein and erythrocyte sedimentation rate, were significantly higher than normal in AFOP patients. In accordance with this increased indicators, injured liver functions were common in AFOP patients. Inversely, AFOP patients had worse clinical conditions including anemia and hypoalbuminemia. For pulmonary function testing, AFOP patients showed the pattern of restrictive mixed with obstructive ventilation dysfunction. For high-resolution computerized tomography (HRCT) findings, the most common pattern for AFOP patients was lobar consolidation which was very similar to pneumonia. However, unlike pneumonia, AFOP patients responded well to glucocorticoids. Conclusion: Patients with AFOP manifest as acute inflammatory-like clinical laboratory parameters and lobar consolidation on HRCT, but respond well to steroid.

The Clinical Characteristics and Outcomes of Follicular Bronchiolitis in Chinese Adult Patients

Follicular bronchiolitis (FB) is a rare interstitial lung disease (ILD) and has been reported in diverse clinical contexts. Six FB patients demonstrated by surgical lung biopsy (SLB) were reviewed between 2009 and 2017 from Nanjing Drum Tower Hospital in China. The average age of subjects was 42 years old (range: 31–55 years). The clinical symptoms were very mild. The laboratory findings showed elevated Erythrocyte sedimentation rate (ESR) and serum globulin and anemia. Pulmonary function tests were normal in four cases. Five cases had underlying diseases, such as, Sjo¨gren’s syndrome, multi-centric castlemans’ disease, idiopathic pneumonia with autoimmune features and abscess. Five cases presented as interstitial lung disease (ILD) on chest imaging with centrilobular or peribronchiolar nodules, ground glass opacities, interlobular septal thickening, cysts and bronchiectasis. Isolated mass was in one patient. The histopathology suggested the changes of FB in all subjects. Prednisone and/or cyclophosphamide were used in four cases, one did the surgery and the other was clinically monitored. All cases were alive at the end of follow up. The adult patients of FB usually have mild symptoms, ILD and underlying diseases. The definite diagnosis needs SLB. The prognosis is depended on their underlying conditions.

Clinical features, risk factors, and outcomes of patients with interstitial pneumonia with autoimmune features: a population-based study

To investigate the clinical features, risk factors and outcomes of patients with interstitial pneumonia with autoimmune features (IPAF). A total of 1429 patients with idiopathic interstitial pneumonia (IIP) and undifferentiated connective tissue disease-associated interstitial lung disease (UCTD-ILD) were screened to identify patients who met IPAF criteria. Clinical, serological, and morphological features of patients with IPAF were characterized. Outcomes between patients with IPAF, UCTD-ILD, and IIP who were divided into idiopathic pulmonary fibrosis (IPF) and non-IPF groups were compared using survival as an endpoint. Patients with IPAF were much common in young female and had lower percentage of ever smoking and a significantly shorter survival than those with non-IPAF (P < 0.001). Subgroup analysis revealed that IPAF cohort survival was worse than that in non-IPF (P < 0.001), but better than that in IPF (P < 0.001). In IPAF cohort, the most common systemic symptom and serological abnormality were Raynaud’s phenomenon (12.9%) and ANA ≥ 1:320 (49.2%); the most frequent high-resolution computed tomography (HRCT) pattern was nonspecific interstitial pneumonia (NSIP) (61.6%). Multivariate analysis indicated that several factors including age, smoking history, organizing pneumonia (OP) pattern in HRCT, and anti-RNP positivity were independently associated with significantly worse survival. IPAF had the distinct clinical features and outcomes compared with other groups of ILD. Additional studies should be needed to explore the underlying autoimmune mechanism and to determine risk stratification in future clinical research.

Severe Mediastinal Abscess after Endobronchial Ultrasound with Transbronchial Needle Aspiration

Endobronchial ultrasound with transbronchial needle aspiration (EBUS‐TBNA) is a minimally invasive and safe technique which is universally accepted for the mediastinum and/or hilum lymph node biopsy. Severe infectious complications following EBUS‐TBNA were occasional, but sometimes life‐threatening.[1] Two patients with severe mediastinal abscess after EBUS‐TBNA were treated successfully with surgical drainage in our department, and we reported here to improve our clinical vigilance to this disease.

Radial Probe Endobronchial Ultrasound-guided Lung Biopsy for Histopathological Diagnosis of Cryptogenic Organizing Pneumonia

inTrodUcTion Cryptogenic organizing pneumonia (COP) is a distinct type of idiopathic interstitial pneumonia with a response rate of 65–85% on corticosteroid therapy. The difficulty of COP diagnosis is that the clinical features and the radiological findings are nonspecific. The pathological hallmark of organizing pneumonia (OP) needs to be confirmed. It can also occur in a variety of other interstitial pneumonia, infectious diseases, vasculitis, and so on.[1] To increase the pathological reliability, larger and more tissue samples are required. According to the current classification of interstitial lung disease and guidelines, the surgical lung biopsy is recommended and is considered to be the best way of obtaining a representative lung specimen.[2] However, the invasive diagnostic procedures require general anesthesia and also increase the morbidity and mortality risks; therefore, only few patients undergo such biopsy. Thus, safer and more acceptable methods for identifying COP are urgently needed.

A Tuberous Sclerosis Family with TSC1 (c.1030-1G>A) Mutation Found through a Female Presenting as Multiple Ground Glass Nodules in Chest Computed Tomography Incidentally

Chest computed tomography (CT) screening is becoming more popular in China. Therefore, more and more rare diseases and early stages of lung diseases were found. Here, we reported a case who presented as multiple ground glass nodules incidentally found in chest CT scan who had been suspected as synchronous multiple primary lung cancer (SMPLC) and/or metastatic cancer. She was finally diagnosed as tuberous sclerosis complex (TSC), an autosomal‐dominant disorder characterized by the formation of hamartomatous lesions in the skin, eyes, kidney, and central nervous system.[1,2] Tuberous sclerosis complex 1 (TSC1) gene mutation (c.1030‐1G>A) was found in her and her family members. This is a very rare report in China.