Pulmonary involvement in primary Sjögren's syndrome, as measured by the ESSDAI.

Objective: Systemic features influence disease prognosis and choice of treatment in primary Sjögren's syndrome (pSS). Our aim was to investigate the prevalence of pulmonary involvement in pSS patients and to classify patients according to the pulmonary domain of the EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI).Methods: This retrospective cohort study included consecutive pSS patients, fulfilling American-European Consensus Group/American College of Rheumatology classification criteria, who visited the Department of Rheumatology and Clinical Immunology, University Medical Center Groningen, in 2015. Data on pulmonary complaints and pulmonary tests were obtained from electronic patient records. Pulmonary involvement was recorded if therapy was needed or follow-up was recommended, and when it was possibly or assumed to be related to pSS instead of coincidental factors.Results: Of the 262 included pSS patients, 88 (34%) had pulmonary complaints, mostly cough or dyspnoea on exertion. Pulmonary diagnostics were performed in 225 patients (86%). Pulmonary involvement was present and assumed to be related to pSS in 25 patients (10%) and possibly related to pSS in 14 (5%). Interstitial lung disease (ILD, n = 15), especially non-specific interstitial pneumonia (n = 7), was present most commonly. In total, 16 patients (6%) were scored as low (n = 4), moderate (n = 11), or high activity (n = 1) on the ESSDAI pulmonary domain.Conclusion: In this cross-sectional study in daily clinical practice, pulmonary involvement was present in 10-15% of pSS patients, of which ILD was most common. Of all pSS patients, 6% were scored as active on the pulmonary domain of the ESSDAI.

The spectrum of structural abnormalities on CT scans from patients with CF with severe advanced lung disease.

RATIONALE: In cystic fibrosis (CF), lung disease is the predominant cause of morbidity and mortality. Little is known about the spectrum of structural abnormalities on CT scans from patients with CF with severe advanced lung disease (SALD). No specific CT scoring system for SALD is available. OBJECTIVES: To design a quantitative CT scoring system for SALD, to determine the spectrum of structural abnormalities in patients with SALD and to correlate the SALD system with an existing scoring system for mild CF lung disease and pulmonary function tests (PFTs). METHODS: 57 patients with CF contributed one CT made during screening for lung transplantation. For the SALD system, lung tissue was divided into four components: infection/inflammation (including bronchiectasis, airway wall thickening, mucus and consolidations), air trapping/hypoperfusion, bulla/cysts and normal/hyperperfused tissue. The volume proportion of the components was estimated on a 0-100% scale; mean volumes for the whole lung were computed. Scores were correlated with Brody-II scores and PFTs. RESULTS: The SALD system identified a wide spectrum of structural abnormalities ranging from predominantly infection/inflammation to predominantly air trapping/hypoperfusion. SALD infection/inflammation scores correlated with Brody-II scores (r(s) = 0.36-0.64) and SALD normal/hyperperfusion scores correlated with forced expiratory volume in 1 s (FEV(1); r(s) = 0.37). Reproducibility for both systems was good. CONCLUSIONS: A CT scoring system was developed to characterise the structural abnormalities in patients with SALD. A wide spectrum was observed in SALD, ranging from predominantly air trapping to predominantly infection/inflammation-related changes. This spectrum may have clinical implications for patients with SALD.

Continuously infused carboplatin used as radiosensitizer in locally unresectable non-small-cell lung cancer: a multicenter phase III study.

PURPOSE: To determine the radiosensitizing effect of prolonged exposure of carboplatin in patients with locally unresectable non-small-cell lung cancer (NSCLC). PATIENTS AND METHODS: Patients with histologically proven NSCLC, performance score <2, weight loss <10%, and normal organ functions were randomized between carboplatin 840 mg/m2 administered continuously during 6 weeks of radiotherapy or thoracic radiotherapy alone (both 60 Gy). Toxicity was evaluated with National Cancer Institute Common Toxicity Criteria (NCI CTC) and the Radiation Therapy Oncology Group (RTOG) criteria. Quality of life was measured with European Organization for Research and Treatment of Cancer (EORTC) QLQ-C30/LC13 questionnaires. RESULTS: One-hundred and sixty patients were included. Pathologically confirmed persistent tumor was present in 53% of patients in the combination arm versus 58% in the radiotherapy alone arm (P=0.5). Median survival in the combination arm was 11.8 [95% confidence interval (CI) 9.3-14.2] months and in the radiotherapy alone arm 11.7 (95% CI 8.1-15.5) months; progression-free survival was not different between arms [6.8 and 7.5 months, respectively (P=0.28)]. Acute toxicity was mild, late toxicity was radiation-induced cardiomyopathy (three patients) and pulmonary fibrosis (five patients). Quality of life was not different between arms, but in all measured patients cough and dyspnea improved, pain became less, and slight paresthesia developed 3 months after treatment. CONCLUSION: Addition of continuously administered carboplatin as radiosensitizer for locally unresectable NSCLC does not improve local tumor control or overall survival.

[Fatal anaphylactic reaction after oral acetazolamide (diamox) for glaucoma]. / Fatale anafylactische reactie na inname van acetazolamide (Diamox) wegens glaucoom.

A woman aged 66 was prescribed acetazolamide (Diamox) in the outpatient clinic because of glaucoma. She went into irreversible anaphylactic shock with massive pulmonary oedema, probably due to a cross reaction in sulphonamide allergy. Before prescribing acetazolamide, the physician should inquire about sulphonamide allergy because of the related chemical structure of the substances. Such an allergy should be regarded as a contraindication.

[Primary sarcoma of A. pulmonalis: a rare cause of cor pulmonale]. / Primair sarcoom van de A. pulmonalis: zeldzame oorzaak van cor pulmonale.

A 64-year-old female presented with fever and weight loss, accompanied by normochromic normocytic anaemia with high erythrocyte sedimentation rate. She developed severe cor pulmonale. Ventilation-perfusion scintigraphy revealed a non-matched perfusion defect of the entire right lung with impaired perfusion on the left side. The CT scan of the thorax revealed an intravascular mass which completely blocked the right pulmonary artery with incomplete obstruction of the pulmonary trunk. Treatment with anticoagulants was started but failed. The presumably thrombotic mass was removed by thoracotomy; the histopathological diagnosis was: sarcoma of the pulmonary artery, incompletely resected. Postoperatively, the condition of the patient showed initial improvement, but she later developed fatal mediastinitis. Pulmonary artery sarcoma is a rare cause of cor pulmonale. Diagnosis is usually only established at operation, curative resection is uncommon and the prognosis is therefore poor--most patients die within months after the diagnosis has been made.

Bronchiolitis obliterans organizing pneumonia and rheumatoid arthritis.

Bronchiolitis obliterans, with or without organizing pneumonia, can be a serious and life-threatening complication of rheumatoid arthritis. We describe a case of bronchiolitis obliterans organizing pneumonia in a patient who recently developed rheumatoid arthritis, presenting as a severe respiratory insufficiency. Diagnosis was made by means of open lung biopsy. Treatment with corticosteroids induced a quick response and substantial improvement of the respiratory symptoms. A simultaneous strong rise in titres of serological tests suggests a relationship between the bronchiolitis obliterans organizing pneumonia and the rheumatoid arthritis.

Pharmacokinetics of ipratropium bromide after single dose inhalation and oral and intravenous administration.

Single doses of ipratropium bromide were administered intravenously, orally and by slow inhalation to ten healthy male volunteers. The plasma level after oral administration followed a low but broad plateau persisting for several hours. After i.v. administration the kinetic parameters were: Vc = 25.9 l, V alpha = 13.1 l, V beta = 3.38 l, t1/2 alpha = 3.85 min, t1/2 beta = 98.4 min, AUC = 15.0 h.ng/ml, kel = 11.8 l/h and total clearance is 2325 ml/min. The bioavailability was 3.3% (range 0.9-6.1%) on comparing the plasma AUCs following i.v. and 20 mg oral administration. The cumulative renal excretion (0-24 h) after i.v. administration was compared with that after oral administration and inhalation. Following oral administration, the apparent systemic availability was around 2%, and after inhalation it was 6.9%. In comparison with oral placebo administration, only after i.v. administration was there a significant change in heart rate (from 63.7 to 90.2 beats/min). The systolic blood pressure rose from 115.1 to 119.6 mm Hg and the diastolic blood pressure from 68.3 to 78.3 mm Hg.

Comparative study of the bronchodilating effects of (-)- and (+)-oxyphenonium bromide.

1. Racemic oxyphenonium bromide, its enantiomers and placebo were inhaled by eight adult patients with chronic airflow obstruction, in a four-way double-blind cross-over study. 2. Forced expiratory volume in one second (FEV1) and slow inspiratory vital capacity (VC) were measured during the first 6 h after inhalation. 3. (+)-Oxyphenonium was found to be the active enantiomer (eutomer), providing better bronchodilation than (+/-)-oxyphenonium. 4. The distomer, (-)-oxyphenonium, showed only a slight effect on FEV1 and VC as compared with placebo.