Pirfenidone treatment in idiopathic pulmonary fibrosis: a Saudi experience

Recent trials involving pirfenidone suggest a beneficial effect in the treatment of idiopathic pulmonary fibrosis [IPF]. To report on the efficacy and safety of pirfenidone in the treatment of patients with IPF, at a tertiary care hospital in Saudi Arabia. The study included 58 patients with IPF who were evaluated from March 2012 to March 2013. During the study period, 33 patients received pirfenidone, and the remaining patients [n = 25] served as a control group. Baseline clinical characteristics, physiological parameters and the results of a 36-Item Short Form Health Survey [SF-36] were compared between the groups. Furthermore, we compared changes in forced vital capacity [FVC], diffusion capacity of the lung for carbon monoxide [DLco], six-minute walk distance [6MWD] and SF-36 for both groups during follow-up. The last follow-up period ended in January 2014. There were no significant differences in baseline clinical characteristics between the groups. Furthermore, we found no differences in FVC, DLco and SF-36 during follow-up [median, 12 months]. However, patients receiving pirfenidone treatment were less likely to experience reductions in 6MWD compared with the control group [13% vs. 52%, respectively; P = 0.001]. Although adverse events were more frequently reported by the pirfenidone group compared with the control group [85 vs. 56%, respectively; P = 0.015], these patients did not require discontinuation of treatment. Pirfenidone treatment preserves functional capacity, as reflected by the 6MWD. Adverse events associated with pirfenidone treatment were generally well tolerated by the patients

Pulmonary hypertension in Saudi Arabia: a single center experience

Several international studies have described the epidemiology of pulmonary hypertension [PH]. However, information about the incidence and prevalence of PH in Saudi Arabia is unknown. To report cases of PH and compare the demographic and clinical characteristics of PH due to various causes in a Saudi population. Newly diagnosed cases of PH [defined as mean pulmonary artery pressure > 25 mmHg at right heart cauterization [RHC]] were prospectively collected at a single tertiary care hospital from January 2009 and June 2012. Detailed demographic and clinical data were collected at the time of diagnosis, along with hemodynamic parameters. Of the total 264 patients who underwent RHC, 112 were identified as having PH. The mean age at diagnosis was 55.8 +/- 15.8 years, and there was a female preponderance of 72.3%. About 88 [78.6%] of the PH patients were native Saudis and 24 [21.4%] had other origins. Twelve PH patients [10.7%] were classified in group 1 [pulmonary arterial hypertension], 7 [6.2%] in group 2 [PH due to left heart disease], 73 [65.2%] in group 3 [PH due to lung disease], 4 [3.6%] in group 4 [chronic thromboembolic PH], and 16 [14.3%] in group 5 [PH due to multifactorial mechanisms]. PH associated with diastolic dysfunction was noted in 28.6% of group 2 patients, 31.5% of group 3 patients, and 25% of group 5 patients. These results offer the first report of incident cases of PH across five groups in Saudi Arabia

Chronic obstructive pulmonary disease lost in translation: Why are the inhaled corticosteroids skeptics refusing to go?

A survey of pulmonologists attending a clinical meeting of the Saudi Thoracic Society found that only 55% of responders considered that inhaled corticosteroids [ICS] had a positive effect on quality of life in Chronic Obstructive Pulmonary Disease [COPD]. Why the divergence of opinion when all the guidelines have concluded that ICS improve quality of life and produce significant bronchodilation? ICS unequivocally reduce the rate of exacerbations by a modest 20%, but this does not extend to serious exacerbations requiring hospitalization. Bronchodilatation with ICS is now documented to be restricted to some phenotypes of COPD. Withdrawal of ICS trials reported a modest decline of FEV[1] [<5%] in half the studies and no decline in the other half. In spite of the guidelines statements, there is no concurrence on whether ICS improve the quality of life and there is no conclusive evidence that the combination of long-acting B2 agonists [LABA] with ICS is superior to LABA alone in that regard. The explanation for these inconclusive results may be related to the fact that COPD consists of three different phenotypes with divergent responses to LABA and ICS. Therapy tailored to phenotype is the future for COPD

Interstitial lung diseases in Saudi Arabia: a single-center study

There are relatively few epidemiological studies on interstitial lung disease [ILD] worldwide. To report the incident cases of ILD and compare our data with reports from other populations. Newly diagnosed ILDs were prospectively collected at a single tertiary care hospital from January 2008 to December 2011. Detailed demographic and clinical data were collected at the time of diagnosis, along with the results from diagnostic procedures, including high-resolution computed tomography [HRCT], serological tests, bronchoalveolar lavage [BAL], transbronchial lung biopsy, endobronchial ultrasound-guided transbronchial needle aspiration [EBUS-TBNA] and surgical lung biopsy. A total of 330 cases were included. The mean age was 55.4 +/- 14.9 years. There was a slight predominance of females [202; 61.2%], and the male-to-female ratio was 1:1.37. The most frequent disease was connective tissue disease [CTD]-associated ILD [34.8%], followed by idiopathic pulmonary fibrosis [IPF] [23.3%], sarcoidosis [20%], and hypersensitivity pneumonitis [6.3%]. Non-classifiable ILD was present in 1.8% of the total ILD cases. HRCT was performed in 97.3% of the cases, BAL in 17.5%, transbronchial lung biopsy in 21.8%, EBUS-TBNA in 4.5%, and surgical lung biopsy in 22.7% [38.6% of which were performed among the idiopathic interstitial pneumonia cases]. CTD-ILD and IPF were the most frequently observed ILDs in this Saudi Arabian population. Similarities and differences were found with respect to the previous reports from other countries

Abrupt withdrawal of inhaled corticosteroids does not result in spirometric deterioration in chronic obstructive pulmonary disease: effect of phenotyping?

Some studies show a decline of FEV [1] only one month after withdrawal of inhaled corticosteroids [ICS], while others show no decline. We speculate that the presence of an asthma phenotype in the Chronic Obstructive Pulmonary Disease [COPD] population, and that its exclusion may result in no spirometric deterioration. We performed a prospective clinical observation study on 32 patients who fulfilled the Global Initiative for Chronic Obstructive lung disease definition of COPD [Grade II-IV]. They were divided into two phenotypic groups. 1. Irreversible asthma [A and B] [n = 13]: A. Asthma: Bronchial biopsy shows diffuse thickening of basement membrane [>/= 6.6 microm]. B. Airflow limitation [AFL] likely to be asthma: KCO > 80% predicted if the patient refused biopsy. 2. COPD [A and B] [n = 19]: A. COPD: hypercapneic respiratory failure with raised bicarbonate, panlobular emphysema with multiple bullas, or bronchial biopsy showing squamous metaplasia and epithelial/subepithelial inflammation without thickening of the basement membrane. B. AFL likely to be COPD: KCO < 80% predicted. The asthma phenotype was significantly younger, had a strong association with hypertrophy of nasal turbinates, and registered a significant improvement of FEV [1] [350 ml] vs a decline of - 26.5 ml in the COPD phenotype following therapy with budesonide/formoterol for one year. Withdrawal of budesonide for 4 weeks in the COPD phenotype resulted in FEV [1] + 1.33% [SD +/- 5.71] and FVC + 1.24% [SD +/- 5.32]; a change of <12% in all patients. We recorded no spirometric deterioration after exclusion of the asthma phenotype from a COPD group

Saroidosis-associated pulmonary hypertension: clinical features and outcomes in Arab patients

Pulmonary hypertension [PH] occurs in many patients with interstitial lung disease, including sarcoidosi. We explored the frequency, clinical characteristics and outcomes of PH in Arab patients diagnosed with pulmonary sarcoidosis. A retrospective study in three tertiary hospitals was performed on 96 patients who underwent Doppler echocardiography. Demographic and clinical characteristics, physiological studies and computed tomography [CT] results were collected and compared between patients with and without PH. Twenty [20.8%] patients were found to have PH. Patients with PH were more likely to be sumptomatic [cough, P= 0.008; dyspnea, P=0.04], to have an advanced radiographic stage [P=0.001], and to be receiving systemic therapy [P=0.0011], compared to those without PH. Physiological data including pulmonary function test parameters, arterial blood gas levels and oxygen saturation at rest and after exercise were all significantly lower in patients with PH compared to those without PH. Compariosn of CT patterns between patients with and without PH showed significant differences in the frequencies of ground-glass opacity [61.5 vs. 28.8%, P=0.032] and fibrosis [76.9 vs., 44.2%, P=0.035]. In total, four patients died during the study period, including three with evidence of PH. The frequency of PH in the present study was 20.8%. Clinical, physiologic and radiographic characteristics appeared to differentiate patients with PH from those without PH. The presence of PH contributed to poor outcomes in patients with pulmonary sarcoidosis

six-minute walk test in patients with pulmonary sarcoidosis

The 6-min walk test [6MWT] is a useful tool to assess prognosis and functional impairment in various pulmonary diseases. To evaluate functional capacity during various stages of pulmonary sarcoidosis and develop a scoring system clinical radiological physiological score [CRP] that can potentially be used to assess the functional status among patients with sarcoidosis. We performed a retrospective study on 26 patients diagnosed with pulmonary sarcoidosis from 2001 to 2007. All patients completed the 6MWT. The parameters assessed during the test included spirometry, arterial blood gas, 6-min walk distance [6MWD], Borg dyspnea score, and initial and end oxygen saturation. Females covered a significantly shorter distance than males [343 m [223-389] vs. 416.5 m [352-500]; P < 0.0001]. In addition, females had a significantly lower SpO[2] at the end of the 6MWT than males [90.5 [61-99] vs. 96 [75-98]; P < 0.03]. The 6MWD was inversely correlated with the final Borg score [r = ?0.603, P = 0.004] and the CRP score [r = -0.364, P = 0.047] and positively correlated with forced expiratory volume in 1 s [FEV[1]]% [r = 0.524, P = 0.006] and forced vital capacity [FVC]% [r = 0.407, P = 0.039]. Female gender, FEV[1]%, final Borg score, FVC%, CRP score, and SpO[2] at the end of the 6MWT are associated with reduced 6MWD. It appears that Saudi patients diagnosed with sarcoidosis have a markedly reduced walking distance compared with other races. The effect of race and ethnicity and the utility of the CRP score as a potential marker to assess functional status require further exploration

Clinical characteristics and computed tomography findings in Arab patients diagnosed with pulmonary sarcoidosis

Sarcoidosis is prevalent worldwide with significant heterogeneity across different ethnic groups. We aimed to describe the clinical characteristics and computed tomography findings among Arab patients with pulmonary sarcoidosis. A retrospective study of patient demographics, symptoms, co-morbid illness, sarcoidosis stage, treatment, pulmonary function and CT results. Of 104 patients, most [77%] were 40 years of age or older at diagnosis, and females in this category [>/= 40 years] significantly outnumbered male patients [69/104 [66.3%] vs. 35/104 [33.7%], P=.003]. The most common complaints were dyspnea [76%], cough [72.1%] and weight loss [32.7%]. The majority of patients displayed impairment in lung function parameters at presentation. However, significant impairment in forced vital capacity, percentage predicted [FVC%] [<50%] was present in only 17% of patients. The most frequent CT finding was mediastinal lymph node enlargement in 49 patients [73.1%]. Parenchymal abnormalities indicating lung fibrosis were noted in 31 patients [46.3%], and traction bronchiectasis was the most common [35.8%] fibrotic pattern detected on CT scans. At presentation, clinical manifestations of sarcoidosis among this sample of Arab patients were similar to reports from other nations. Further studies are needed to explore the effects of race and ethnicity on disease severity in the Middle East