Rituximab therapy in a patient with steroid-refractory bird fancier’s lung

A 62-year-old woman presented with a 3-month history of shortness of breath on exertion and dry cough. On examination, she was noted to have fine end-inspiratory crepitations over the upper zone of the lungs. Pulmonary function tests (PFTs) showed a restrictive defect. Initial chest radiography revealed diffuse reticular interstitial shadowing while high-resolution CT scan of the thorax showed fibrotic changes. Avian precipitins were also highly positive for pigeons, parrots and budgerigars. Taking into account these results, the patient was diagnosed with hypersensitivity pneumonitis. Antigen avoidance, oral glucocorticoids and azathioprine achieved an initial improvement in PFTs and symptoms; however, the patient still deteriorated, requiring long-term oxygen therapy. While working the patient up for lung transplantation, rituximab was given to good effect (acting as a bridging therapy) as it achieved symptomatic relief and stabilisation of her PFTs.

Pleuroparenchymal fibroelastosis (PPFE) treated with lung transplantation and review of the literature

A 26-year-old woman presented with a 15-year history of non-progressive dyspnoea. Chest imaging showed bilateral apical pleural and parenchymal scarring, pleural thickening and bronchiectasis. Pulmonary function tests showed a moderate restrictive defect. Non-invasive workup was non-revealing; therefore, the patient was referred for video-assisted thoracic surgery and lung biopsy. Histopathology revealed pleural thickening and, subpleural parenchymal fibrosis and elastic tissue deposition. Lung parenchyma further away from the pleura was well preserved. Based on these findings, the patient was diagnosed with pleuroparenchymal fibroelastosis (PPFE). Since PPFE is a progressive disorder without effective medical therapies, and given our patient’s worsening symptoms, she underwent bilateral lung transplantation. It has been almost 4 years since the lung transplantation, our patient continues to do well. To the best of our knowledge, to date, this is the longest follow-up reported for a PPFE patient undergoing lung transplantation.

Atrial septal defect morphology and stenting in hypoplastic left heart syndrome after hybrid palliation

AimsThe aim of this study was to describe atrial septal defect morphology in hypoplastic left heart syndrome, to report the incidence of restrictiveness and its relationship with defect morphology, to correlate restriction with midterm outcome, and to describe our interventional approach to restrictive defect.Methods and resultsFrom 2011 to 2015, 31 neonates with hypoplastic left heart syndrome underwent hybrid procedure with pulmonary artery banding and ductal stenting at our Institution. Restrictive physiology of the atrial septal defect was based on Doppler gradient >6 mmHg through the defect and on clinical signs of pulmonary hypertension. The mean gradient was then measured invasively. Restrictive defect occurred in 11/27 patients (40%). The restrictive group showed three ostium secundum defects (27%) and eight complex morphologies (73%). Conversely, in the non-restrictive group, we observed 11 ostium secundum defects (69%) and five complex morphologies (31%). Early balloon atrioseptostomy was required in three cases. Late restriction occurred in eight patients and was dealt with balloon dilation, stenting, or atrioseptectomy. There was no significant difference between restrictive and non-restrictive groups in terms of early or 12-month survival.ConclusionsComplex morphologies were more frequently related to restrictiveness. Stenting technique has a crucial role, as the procedure carries a significant risk for stent migration. Effective treatment of restrictive atrial septal defect is related to a better outcome, as it leads to equalisation of survival between patients with and those without restrictive atrial septal defect.

Bronchial Responsiveness in Patients with Restrictive Spirometry

Background. Improvement in PFT after bronchodilators is characteristic of obstructive airway diseases such as COPD. However, improvement in patients with restrictive pattern is occasionally seen. We aim to determine the clinical significance of a bronchodilator responsive restrictive defect.Methods. Patients with restrictive spirometry and a bronchodilator study were identified at the University of Oklahoma and Oklahoma City VAMC between September 2003 and December 2009. Restriction was defined as a decreased FVC and FEV1, with normal FEV1/FVC. Responsiveness to bronchodilators was defined as an improvement in FEV1 and/or FVC of at least 12% and 200 mL. Patients with lung volume measurements had their clinical and radiographic records reviewed.Results. Twenty-one patients were included in the study. Most were current or ex-smokers, with most being on bronchodilators. The average FVC and FEV1 were65±11%and62±10%of the predicted, respectively. Most patients (66%) had a normal TLC, averaging90±16%of the predicted. RV, RV/TLC, and the TLC-VA values strongly suggested an obstructive defect.Conclusions. Reversible restrictive pattern on spirometry appears to be a variant of obstructive lung disease in which early airway closure results in air trapping and low FVC. In symptomatic patients, a therapeutic trial of bronchodilators may be beneficial.

Postural changes in lung volumes and respiratory resistance in subjects with obesity

Reduced functional residual capacity (FRC) is consistently found in obese subjects. In 10 obese subjects (mean ± SE age 49.0 ± 6 yr, weight 128.4 ± 8 kg, body mass index 44 ± 3 kg/m2) without respiratory disease, we examined 1) supine changes in total lung capacity (TLC) and subdivisions, 2) whether values of total respiratory resistance (Rrs) are appropriate for mid-tidal lung volume (MTLV), and 3) estimated resistance of the nasopharyngeal airway (Rnp) in both sitting and supine postures. The results were compared with those of 13 control subjects with body mass indexes of <27kg/m2. Rrs at 6 Hz was measured by applying forced oscillation at the mouth (Rrs,mo) or the nose (Rrs,na); Rnp was estimated from the difference between sequential measurements of Rrs,mo and Rrs,na. All measurements were made when subjects were seated and when supine. Obese subjects when seated had a restrictive defect with low TLC and FRC-to-TLC ratio; when supine, TLC fell 80 ml and FRC fell only 70 ml compared with a mean supine fall of FRC of 730 ml in control subjects. Values of Rrs,mo and Rrs,na at resting MTLV in obese subjects were about twice those in control subjects in both postures. Relating total respiratory conductance (1/Rrs) to MTLV, the increase in Rrs,mo in obese subjects was only partly explained by their reduced MTLV. Rnp was increased in some obese subjects in both postures. Despite the increased extrapulmonary mass load in obese subjects, further falls in TLC and FRC when supine were negligible. Rrs,mo at isovolume was increased. Further studies are needed to examine the causes of reduced TLC and increases in Rrs,mo and sometimes in Rnp in obese subjects.

Fibrothorax and Severe Lung Restriction Secondary to Lupus Pleuritis and Its Successful Treatment by Pleurectomy

Pleural disease is a common pulmonary manifestation of systemic lupus erythematosus (SLE) that usually responds to corticosteroids and other immunosuppressive agents. In the present report, a new approach, pleural decortication, was used in a patient with medically refractory chronic pleuritis secondary to severe SLE. A 26-year-old woman with known SLE developed progressive dyspnea and pleuritic chest pain over several months. The other systemic manifestations of her lupus were controlled with cyclophosphamide and prednisone. A computed tomography scan revealed a persistent, small, loculated right pleural effusion; pleural thickening; and atelectasis of the right middle and lower lobes. Pulmonary function tests showed a severe restrictive defect. The patient was disabled by her severe dyspnea despite maximal medical therapy, and, therefore, surgery was considered. A right thoracotomy revealed entrapment of the right lung by dense visceral pleura. Decortication was performed. On pathology, pleuritis with vascular pleural adhesions was found. No lupus pneumonitis was noted. Postoperatively, a significant clinical improvement in dyspnea was evident within several weeks. On a 6 min walk test, the patient achieved 384 m with a Borg dyspnea scale rating of 2 compared with 220 m and a Borg dyspnea scale rating of 4 preoperatively. Her forced vital capacity improved from 24% predicted to 47% predicted, and her total lung capacity improved from 35% predicted to 54% predicted. Medical therapy of systemic lupus erythematosus has been proven to be effective in controlling pleuritis in most cases. However, in the event of refractory pleuritis or pleural thickening, decortication may be a viable alternative.