Elena Torricelli

Atypical HRCT manifestations of pulmonary sarcoidosis

PurposeTo present our experience of cases of pulmonary sarcoidosis with atypical HRCT patterns found during 2016 focusing on the differential diagnosis to contribute to the difficult role of the radiologist in the disease identification and to help the clinicians to reach the diagnosis.Materials and methodsThe HRCT examinations of 47 patients with sarcoidosis were studied retrospectively. All patients had a histopathological confirm of the disease. 29 (61.7%) show a typical pulmonary pattern and 18 (38.3%) an atypical pattern. The latter were evaluated by three experienced radiologists dedicated to thoracic disease to radiologically define the predominant pattern of presentation.ResultsIn the 18 patients (38.3%) with atypical sarcoidosis, the following parenchymal patterns were observed: four patients (22.2%) had interstitial fibrotic alterations, three patients (16.6%) with reticular pattern with inter–intralobular septal thickening, two patients (11.1%) with small-airway involvement with mosaic oligoemia, two patients (11.1%) with pleural involvement (pneumothorax and pleural plaques), one patient (5.5%) with fibrocystic changes, 1 (5.5%) with halo-sign, 1 (5.5%) with diffuse bilateral ground-glass opacities, and 1 (5.5%) with isolated lung mass; in addition, three patients (16.6%) with atypical lymph node pattern were also found.Conclusions and DiscussionThe atypical pulmonary alterations found in CT examination can be confused with other lung diseases and they are always a challenge even for the most experienced radiologist. In our experience, cases with atypical pulmonary sarcoidosis patterns evaluated in the study are consistent with similar cases described in the literature, both in lymph node and atypical parenchymal involvement. All the atypical characteristics of the work should alert the radiologist to consider sarcoidosis among the possible differential diagnoses, always correlating the results of the computed tomography examination with appropriate clinical-laboratory evaluations.

A group of patients with Marfan’s syndrome, who have finger and toe contractures, displays tendons’ alterations upon an ultrasound examination: are these features common among classical Marfan patients?

AbstractThe involvement of the musculoskeletal system with other mild pleiotropic manifestations represents a clinical criterion, called “systemic features,” to d iagnose Marfan’s syndrome. We aimed to investigate the features of the hands and feet redressable contractures present in a group of Marfan patients. In 13 patients with previously diagnosed Marfan’s syndrome, an accurate clinical examination was performed. In particular the characterization of the musculoskeletal system by visual analogic scale to measure muscle pain (VAS) and muscle strength (MRC system) was carried out; the Beighton scale score was used to evaluate the articular hypermobility. Ultrasound examination (US) was performed to detect deep-superficial flexor tendons and extensor tendons of both hands, and the short and long flexor and extensor tendons of the fingers and toes in static and dynamic positions. The ImageJ program was adopted to measure a profile of tendon echo-intensity. A reduction of the thickness of all tendons was detected by US in our patients; the VAS and Beighton scale scores were in normal ranges. The profile of tendon echo-intensity showed different textural details in all Marfan patients. This study provides evidence for other contractures’ localization, and for altered findings of the tendons in patients with Marfan syndrome and finger/toe contractures. These changes may be associated with structural modifications in connective tissue.

Airway-Centered Pleuroparenchymal Fibroelastosis Associated with Non-Necrotizing Granulomas: A Rare New Entity

Pleuroparenchymal fibroelastosis is a rare form of upper-lobe-dominant progressive pulmonary fibrosis characterized histologically by visceral pleural thickening with collagenous fibrosis, subpleural elastosis, and intra-alveolar collagenous fibrosis. It was first described 25 years ago by Amitani et al. This report firstly describes a new variant or rare phenotype of PPFE with airway involvement, minimal pleuroparenchymal connections, and non-necrotizing granulomas.

Pulmonary hypertension nosography: are all patients classifiable?

Elena Torricelli, Chiara Cresci A 73-year-old never smoking woman was admitted for exertional dyspnoea to the Respiratory Medicine Section of our hospital in June 2014. The patient’s past clinical history revealed systemic arterial hypertension, persistent atrial fibrillation, non-insulin dependent diabetes, hypothyroidism, and hypercholesterolemia. She was taking in treatment with acenocoumarol and metformin. The patient had progressive exercise dyspnoea (NYHA class II) and ankle swelling since 2011. She had been diagnosed with interstitial lung disease with pulmonary hypertension (PAPs of 60 mmHg by transthoracic echocardiography) and hypoxemic respiratory insufficiency. She was referred to our hospital following further worsening of dyspnoea and reduction in exercise tolerance despite long-term oxygen therapy (1 L/min). At admission, she had dyspnoea on minor activities (modified Medical Research Council— mMRC = 3, NYHA class III), cyanosis, increased respiratory rate (22 breaths/min), and ankle swelling. She had systemic pressure at lower limits of normality (105/ 65 mmHg) and a BMI of 28 (158 cm, 71 kg). At physical examination, fine crackles were audible at both lung bases. She had low (54 mmHg) arterial oxygen tension in oxygen therapy with 1 L/min with CO2 tension of 50 mmHg, in a framework of compensated respiratory acidosis (HCO3 33 mmHg). No alterations of serum electrolytes were found. We got sufficient correction of hypoxia (PaO2 63 mmHg) by increasing flow oxygen support at 3 L/min with a modest increase of hypercapnia (PaCO2 54 mmHg). The chest radiograph showed increased cardiac size, bulging of the second arch on the left mediastinal border compatible with enlargement of the pulmonary artery common trunk (Fig. 1). No signs of pulmonary congestion were observed. The electrocardiogram showed atrial fibrillation with average ventricular rate of 77 beats per minute, intraventricular conduction delay, and right axial deviation. Transthoracic echocardiography showed severe tricuspidal regurgitation with TAPSE (tricuspid annular plane systolic excursion) of 15 mm, PAPs of 70 mmHg, right and left atrial enlargement, interventricular septum paradoxical movement, inferior vena cava dilatation with no change on inspiration, mild pericardial effusion, and normal size and wall thickness of the left ventricle, without alteration on the segmental kinesis. Cardiac ejection fraction was preserved (61%). No diastolic relaxation abnormalities were detected. E/A ratio [early transmitral flow (E) and the late flow with atrial contraction (A)] was 0.8 and the ratio E/e0 [early filling velocity on transmitral Doppler (E) with the early relaxation velocity on tissue Doppler (e0)] was 7. Only mild mitral regurgitation was described. Calcification of the cusps of the aortic valve with normal opening was described. Aortic valve peak gradient was 22 mmHg. We decided to treat the patient with furosemide 20 mg iv TID, oxygen therapy (3 L/min) with a partial clinical & Elena Torricelli e.torricelli@alice.it

Fibrotic Lung Toxicity Induced by Hydroxycarbamide

A patient treated for 4 months with hydroxycarbamide (hydroxyurea) for chronic myelomonocytic leukemia was admitted to hospital for recently developed severe dyspnea and acute respiratory failure. The computed tomographic scan of the chest showed diffuse ground glass opacities, some centrilobular low-density nodules (resembling hypersensitivity pneumonitis-like pattern), and minimal interstitial reticulation of the subpleural region. The analysis of bronchoalveolar lavage fluid excluded infection, as did serological examinations. The patient was started on oxygen therapy and with relief of thrombocytopenia and suspected hemolytic anemia, hydroxyurea treatment was discontinued. The patient underwent steroid therapy, with a rapid progressive improvement of clinical and radiological features. As hydroxyurea is increasingly used for a number of systemic disorders, physicians must be aware of its potential lung toxicity, requiring immediate cessation of the treatment and empiric corticosteroid therapy.

Endoscopic treatment with endobronchial valves of Persistent Air Leak (PAL)

Introduction: Persistent air leak is defined as prolonged (>5 days) need for a chest drain after pneumothorax, chest trauma or lung resection (28% to 60%). Standard treatment consists in maintaining the chest tube with or without suction. Pleurodesis, pleural decortication or surgical resection are further options. PAL occurrence extends the average hospital stay and increases costs. Aims and objectives: We evaluated safety and efficacy of endoscopic treatment of PAL with endobronchial valves. Methods: We reviewed the files of all patients treated for PAL during 6 years in 2 Italian Interventional Pulmonology Center. Results: Since September 2011 25 patients underwent endoscopic treatment of PAL due to: lung resection (9, 7 cancer), pneumothorax (11, 6 spontaneous-5 iatrogenic) or pleural empyema (5). Treatment was performed 17.5±10 days after chest tube insertion through flexible (92%) or rigid bronchoscopy (2 pt). Air leaks were located at a segmental (11pt) or lobar (12 pt) level using an Olympus balloon; in 2 patients 2 adjacent lobes were excluded. We used Zephyr Endo Bronchial Valves (6 pt) or Olympus Spiration Valve System (17 pt) or both (2 pt). Bronchoscopies lasted 28.6±11.5 minutes, air leak resolved immediately in 23 pt out of 25 (92%). No severe complications were described and pneumothorax never recurred. Conclusions: This retrospective study suggests that bronchoscopic treatment of PAL with bronchial valves is a minimally invasive procedure, effective and safe, regardless of its cause. Future prospective studies should focus of cost/effectiveness compared to conventional treatment.