Restrictive Pulmonary Function Tests Research Articles

Cluster Analysis to Identify Long COVID Phenotypes Using 129Xe Magnetic Resonance Imaging: A Multi-centre Evaluation.

Long COVID impacts ∼10% of people diagnosed with COVID-19, yet the pathophysiology driving ongoing symptoms is poorly understood. We hypothesised that 129Xe magnetic resonance imaging (MRI) could identify unique pulmonary phenotypic subgroups of long COVID, therefore we evaluated ventilation and gas exchange measurements with cluster analysis to generate imaging-based phenotypes. COVID-negative controls and participants who previously tested positive for COVID-19 underwent 129XeMRI ∼14-months post-acute infection across three centres. Long COVID was defined as persistent dyspnea, chest tightness, cough, fatigue, nausea and/or loss of taste/smell at MRI; participants reporting no symptoms were considered fully-recovered. 129XeMRI ventilation defect percent (VDP) and membrane (Mem)/Gas, red blood cell (RBC)/Mem and RBC/Gas ratios were used in k-means clustering for long COVID, and measurements were compared using ANOVA with post-hoc Bonferroni correction. We evaluated 135 participants across three centres: 28 COVID-negative (40±16yrs), 34 fully-recovered (42±14yrs) and 73 long COVID (49±13yrs). RBC/Mem (p=0.03) and FEV1 (p=0.04) were different between long- and COVID-negative; FEV1 and all other pulmonary function tests (PFTs) were within normal ranges. Four unique long COVID clusters were identified compared with recovered and COVID-negative. Cluster1 was the youngest with normal MRI and mild gas-trapping; Cluster2 was the oldest, characterised by reduced RBC/Mem but normal PFTs; Cluster3 had mildly increased Mem/Gas with normal PFTs; and Cluster4 had markedly increased Mem/Gas with concomitant reduction in RBC/Mem and restrictive PFT pattern. We identified four 129XeMRI long COVID phenotypes with distinct characteristics. 129XeMRI can dissect pathophysiologic heterogeneity of long COVID to enable personalised patient care.

The clinical, functional, and radiological effect of long-term used immunosuppressive therapy for post-COVID-19 interstitial lung disease.

Persistent interstitial lung disease (ILD) after COVID-19 infection can lead to severe loss of respiratory function and a decrease in the quality of life. There is no consensus regarding the treatment of post-COVID-19-ILD. This study aims to investigate the effectiveness of immunosuppressive treatment for this group of patients. This retrospective observational study included patients diagnosed with post-COVID-19-ILD from 2021 to 2022. Patients who had pulmonary symptoms, required prolonged oxygen therapy, and/or had restrictive pulmonary function test (PFT) and/or DLCO <80%, with diffuse parenchymal involvement on high-resolution computed tomography (HRCT), were given immunosuppressive treatment with methylprednisolone and/or mycophenolate mofetil (MMF) and followed up for 6 months. Among the 48 patients, 35 were treated. Two patients were excluded due to discontinued treatment and passed away before the study period ended. Of 33 cases, 21 (66.6%) were treated with methylprednisolone, 11 (33%) with methylprednisolone + MMF, and 1 (0.4%) with MMF alone. Comparing baseline and 6th-month data revealed significant improvement in mMRC score, saturation (SpO2), FVC, FVC%, FEV%, and DLCO% values (p<0.005). While regression was observed in all radiologic findings, regression in ground glass and reticulation was statistically significant (p<0.005). When the 1st and 6th-month data were compared, a significant increase was observed in SpO2 and DLCO% values (p=0.016) and there was a significant regression in reticulation radiologically (p=0.01). Long-term immunosuppressive therapy may be preferred in proper cases of post-COVID-19-ILD as an effective and safe treatment option that improves the quality of life, respiratory parameters, and radiologic findings.

The clinical use of impulse oscillometry in neuromuscular diseases

BackgroundThe patients with neuromuscular diseases (NMD) are very fragile and it is hard to evaluate respiratory involvement of the primary disease in this group. Therefore, our study aimed to reveal the relationship between pulmonary function tests (PFT) and impulse oscillometry (IOS) and their correlation with respiratory clinical findings in NMD. Material and methodsA total of 86 consecutive patients with NMD were included. The clinical findings of respiratory involvement, PFT, and IOS results of the patients were analyzed. ResultsForty patients out of 86 were female. There were 29 patients with amyotrophic lateral sclerosis, four patients with myasthenia gravis, and 53 patients with muscular dystrophies/myopathies. According to the PFT results, 47 patients had restrictive PFT. However, there was no difference in IOS parameters when we compared the patients according to restrictions in PFT. A positive correlation was found with FVC %pred and X5. PEF %pred values were positively correlated with X10, X15, and X20, and negatively correlated with AX and R5-20. The patients with worse swallowing capability had increased Rrs levels, and more negative Xrs levels. The shortness of breath led to lower FEV1 %pred., higher R5, AX and R5-20, and also more negative X10, X15, and X35. ConclusionClinically reported dysphagia, a decreased capability of coughing, and shortness of breath in patients with NMD make Rrs increase in general, but Xrs parameters, which mainly express rib cage elasticity, turn more negative. In patients with NMD, IOS monitoring may help in evaluating the regression in respiratory functions, however, future studies are needed to understand more.

Relation between pulmonary function tests and ultrasonographic changes of asymptomatic anterior chest wall joints in rheumatoid arthritis patients

BackgroundUltrasonography can detect different changes in anterior chest wall (ACW) joints in patients with rheumatoid arthritis (RA) even before being clinically manifested. Airways, pleura, lung parenchyma, and vascular compartment all may be attacked by RA. This study was aiming at detecting the relation between ultrasonographic changes of asymptomatic ACW joints and pulmonary function tests (PFTs) in patients with RA.ResultsUS detected subclinical changes of ACW joints in (74.2%) of RA patients with significant difference between total US changes in RA (74.2%) and control (21.2%) (p < 0.001). MSJ ankylosing and erosions were highly associated with limited chest expansion in RA group (P <0.001). Restrictive PFTs were associated with SCJ synovitis (p < 0.05), SCJ PD activity (p < 0.05), SCJ erosions (0.02), and highly associated with MSJ ankylosing and erosions (p < 0.001).ConclusionsThis study demonstrated that ultrasonographic subclinical changes in ACW joints are associated with restrictive pattern by spirometry and limited chest expansion in RA patients.Trial registrationClinicalTrials.gov Identifier: NCT05119491. Registered 15 November 2021—retrospectively registered.

Characteristics of musculoskeletal ultrasound and its relationship with systemic inflammation in systemic sclerosis patients

Aim of the workTo describe musculoskeletal ultrasound (MSUS) findings in patients with systemic sclerosis (SSc) and to study their relation with cardiopulmonary and skin affection. Patient and methodsIn 50 SSc patients, hands, wrists and six entheses sites were evaluated by MSUS. Entheses were scored using the Madrid Sonography Enthesitis Index (MASEI). The modified Rodnan skin score (mRSS) was assessed. ResultsThey were 42 females/8males with a mean age 36.2 ± 11.6 years, disease duration 3.5 ± 4.2 years, 27 had limited skin affection and 23 diffuse and the mRSS was 4.1 ± 15.3. 54% had arthritis, 10% tendon friction rub, 16% joint contractures and 10% calcinosis. 18% had left ventricular hypertrophy, 34% had pulmonary hypertension, 20% had restrictive pulmonary function test (PFT) and 24% had interstial lung disease (ILD). Synovitis was detected in 58% and was significantly related to mRSS, left ventricular hypertrophy, PFT, pulmonary hypertension and ILD (p = 0.023, p = 0.03, p = 0.006, p = 0.026 and p = 0.039 respectively). Common extensor tenosynovitis was detected in 82% and was significantly related to mRSS, PFT and ILD (p = 0.019, p = 0.043 and p = 0.032 respectively). Joint erosions were detected in 36% and were significantly related to age and C-reactive protein (CRP) (p = 0.01 and p = 0.034). Enthesopathy showed a significant relation with erythrocyte sedimentation rate (ESR), CRP and mRSS (p = 0.014, p = 0.013, p = 0.025, respectively). ConclusionArticular involvement is common in SSc and underestimated by clinical examination. Synovitis is associated with cardiovascular complication in SSc. Tenosynovitis is clinically related to restrictive pulmonary function. Enthesopathy should be kept in mind in symptomatic patients with systemic sclerosis.

Frequency of restrictive pulmonary function tests in patients with chronic Hepatitis C

Hepatis C infection is a common health problem world wide and is the major cause of chronic liver disease in Pakistan. Common complications of chronic hepatitis c infection are cirrhosis, ascities and hepatocellular carcinoma. Also HCV infection is associated with several extra hepatic manifestations including interstitial lung fibrosis. It has been found that frequency of pulmonary fibrosis is increased in patients with cirrhosis of liver as the stage of cirrhosis advances.This lung fibrosis can cause restrictive pattern of pulmonary function tests. This study was conducted to determine frequency of restrictive pulmonary function in patients with different stages of chronic hepatitis C infection, based on child pough classification.&#x0D; Study design&#x0D; This is descriptive case study&#x0D; Method&#x0D; 99 patients of age range 20 to 80 year, both males and females having chronic hepatitis C infection and interferon based treatment naïve patients were included from outpatient and inpatient department were selected. Patients were divided into three groups according to child’s criteria i.e. group A, group B, and group C. Pulmonary function tests were performed on patients in all three groups to look for FEV1/FVC ratio in all patients. FEV1/FVC ratio more than 80 was considered as restrictive pulmonary function.&#x0D; Results&#x0D; Out of 99 patients 32(35%) were found to have restrictive pattern of pulmonary functions&#x0D; Conclusion&#x0D; Chronic hepatitis C infection is associated with restrictive pattern of pulmonary function, suggestive of pulmonary fibrosis.

Study of pulmonary function tests in multitransfused children with beta thalassemia major: an observational study

Background: Thalassemia is a hemoglobinopathy requiring multiple blood transfusions leading to iron deposition in various organs. Abnormalities in pulmonary function in thalassemia major patients have been found in various studies. Our aim was to study pulmonary function tests (PFT) in multitransfused children with beta thalassemia major.Methods: PFT was done in forty beta thalassemia major (TM) patients and forty normal healthy age and sex matched individuals and the results were analyzed.Results: Out of 40 thalassemia major patients, 20 (50%) had normal PFT and the rest 20 (50%) had an abnormal PFT out of which 18 (90%) had restrictive PFT and 2 (10%) had obstructive PFT. Out of 40 controls, all 40 (100%) had normal PFT.Conclusions: Alteration of pulmonary function can be seen even in well-chelated TM patients. The respiratory system should be evaluated annually by PFTs even in asymptomatic patients to prevent pulmonary sequelae.

Viral hepatitis and interstitial lung diseases: can HRCT assess their relation and characterize its pattern?

BackgroundClinical and pathological studies suggested the presence of a relation between active viral hepatitis and interstitial lung diseases (ILD) ranging from mild to moderate relation. Most studies attribute this relation to viral geno-physiological characteristics. The purpose of the study is first to assess the role of high-resolution computed tomography (HRCT) in evaluating the relation between viral hepatitis and ILDs, then to characterize the predominant pattern of ILD that affects patient management and prognosis.ResultsThis prospective study was conducted on 300 patients with viral hepatitis. They were divided into three groups of patients according to blood viremia assessed by polymerase chain reaction (PCR) as well as the diffusing capacity of carbon monoxide (DlCO) in examined pulmonary function tests (PFT). Group [A] included 100 patients with low or moderate viremia and showing normal or low to moderate DlCO decline (> 50%). Group [B] included 100 patients with high viremia and showing normal or low to moderate DlCO decline (> 50%). Group [C] included 100 patients with high viremia and showing a restrictive DlCO decline pattern (< 50%). The study included 182 males and 118 females with a ratio of 3:2, while the age ranged between 40 and 70 years (mean age of 55 years). No ILD was found among the group [A] patient. Meanwhile, 27% of patients in the group [B] showed a non-fibrotic pattern of ILD and 50% of patients in the group [C] showed a fibrotic pattern of ILD. Among patients in group [B] and group [C] together, 77 patients showed ILD accounting for 38.5%.ConclusionIn concordance with the results of the previous clinicopathological and geno-physiological studies, our HRCT results further established a mild to moderate relation between active hepatitis and ILD regardless of the pulmonary functions. The fibrotic pattern of ILD with poor response to therapy and poor prognosis was found in those patients with concomitant restrictive PFT and rapidly progressive symptoms.

AN UNUSUAL CASE OF DESQUAMATIVE INTERSTITIAL PNEUMONIA RECURRENCE AFTER LUNG TRANSPLANT

SESSION TITLE: Tuesday Fellows Case Report Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/22/2019 01:00 PM - 02:00 PM INTRODUCTION: Desquamative interstitial pneumonia (DIP) is a form of interstitial lung disease that is characterized by diffuse interstitial infiltrates on chest X-ray, restrictive pulmonary function tests and intra-alveolar accumulation of macrophages. We report a rare case of DIP recurrence after lung transplantation complicated by Aspergillus and Cytomegalovirus (CMV) infection. CASE PRESENTATION: The patient is a 59 year-old male former smoker with a history of Hepatitis C treated in 2006 with Ribavirin and Interferon. He was evaluated for progressive hypoxic respiratory failure requiring bilateral lung transplantation in December 2017. Explanted lung pathology was consistent with end-stage DIP. Post-surgical course was complicated with antibody-mediated rejection requiring intravenous immunoglobulin therapy and plasmapheresis, Aspergillus pneumonia and Cytomegalovirus (CMV) viremia. 14 months later, he presented to hospital with dyspnea and cough. He was hypoxic requiring 2L/min at rest. Computed tomogram (CT) of the chest showed diffuse ground glass opacities bilaterally and a new patchy left basilar airspace opacity. His bronchoscopy and transbronchial biopsy showed a DIP-like injury pattern, focal interstitial fibrosis and chronic bronchiolitis. Respiratory cultures grew Pseudomonas putida for which IV Piperacillin and Tazobactam was given. Laboratory studies showed speckled pattern ANA with a titre of 1:160 and negative anti Ds-DNA, RF, anti-CCP, anti ScL-70, ANCA, SSA/SSB and CPK. HCV RNA was undetected with normal cryoglobulin levels. While he responded to Medrol 32 mg daily and was discharged home without oxygen supplementation, the mechanism for the DIP-like pattern is not well understood. His immunosuppressive regimen post lung transplantation was tacrolimus and mycophenolate. DISCUSSION: In our patient, DIP recurrence post lung transplantation suggests presence of a systemic disease. Though the patient did not show any evidence of connective tissue disease, further workup is pending. The reported association between history of Hepatitis C and DIP in previous case reports may explain this patient’s presentation, however undetected HCV RNA with normal liver function would not support this theory. It is also possible that Aspergillus and CMV infection may have triggered a DIP-like pattern as described in another case post lung and renal transplantation. To our knowledge, this is the second reported case of DIP recurrence after lung transplant. Early recognition and treatment of DIP is essential as it has the most favorable prognosis of all Idiopathic Interstitial Pneumonia subtypes. In cases where DIP occurs in lung transplant patients, opportunistic pathogens might be suspected as causative agents. CONCLUSIONS: It is important to diagnose and treat the underlying etiology that causes DIP so that recurrence after lung transplantation can be prevented. Reference #1: King, M. B., et al. (1997). "Recurrence of desquamative interstitial pneumonia after lung transplantation.” Am J Respir Crit Care Med 156(6): 2003-2005. DISCLOSURES: No relevant relationships by alaa abu-sayf, source=Web Response No relevant relationships by Bathmapriya Balakrishnan, source=Web Response No relevant relationships by Aditya Kotecha, source=Web Response no disclosure on file for Lisa Stagner; No relevant relationships by Krishna Thavarajah, source=Web Response

Chest CT Has Prognostic Value at BOS Diagnosis after Lung Transplantation

Purpose Long-term survival after lung transplantation (LTx) is hampered by chronic lung allograft dysfunction, with bronchiolitis obliterans syndrome (BOS) as its most common phenotype. Bronchiectasis (BRECT), hyperinflation and airtrapping are considered the key features of BOS on chest CT. We investigated whether chest CT and key features have prognostic value at BOS diagnosis in patients with established BOS after LTx. Methods Double LTx recipients transplanted between 2004-2015 with a survival of >90 days (n=668) who developed BOS (n=118) were included. BOS was defined as a persistent FEV₁ decline of ≥20%, in absence of other conditions explaining FEV₁ decline, restrictive pulmonary function tests or persistent infiltrates. Chest CT was scored at BOS diagnosis by a radiologist experienced in LTx imaging with an adapted Brody score (1 score for the whole lung) and subscores (BRECT score, hyperinflation score, mucous plugging score, peribronchial thickening score and parenchyma score). Post-BOS survival of patients with a score higher than the median was compared with post-BOS survival of patients with a score lower than the median. Results Patients with a higher adapted Brody score (n=38), demonstrated worse survival compared to patients with a lower adapted Brody score (n=52, p=0.019) (figure). Additionally, patients with a higher mucous plugging score (n=19), peribronchial thickening score (n=32) and parenchyma score (n=70), had worse survival compared to patients with lower mucous plugging score (n=99, p=0.0003), peribronchial thickening score (n=86, p=0.0046) and lower parenchyma score (n=48, p=0.016). In contradiction, there was no significant difference in post-BOS survival between patients with a higher or lower BRECT score (p=0.34) and hyperinflation score (p=0.30). Conclusion Chest CT, and in particular the adapted Brody score, mucous plugging score, peribronchial thickening score and parenchyma score, have prognostic value at BOS diagnosis in patients with established BOS after LTx.

High-resolution computed tomography findings in humoral primary immunodeficiencies and correlation with pulmonary function tests.

AIMTo compare high-resolution computed tomography (HRCT) findings between humoral primary immunodeficiencies (hPIDs) subtypes; to correlate these findings to pulmonary function tests (PFTs).METHODSWe retrospectively identified 52 consecutive adult patients with hPIDs who underwent 64-row HRCT and PFTs at the time of diagnosis. On a per-patient basis, an experienced radiologist recorded airway abnormalities (bronchiectasis, airway wall thickening, mucus plugging, tree-in-bud, and air-trapping) and parenchymal-interstitial abnormalities (consolidations, ground-glass opacities, linear and/or irregular opacities, nodules, and bullae/cysts) found on HRCT. The chi-square test was performed to compare the prevalence of each abnormality among patients with different subtypes of hPIDs. Overall logistic regression analysis was performed to assess whether HRCT findings predicted obstructive and/or restrictive PFTs results (absent-to-mild vs moderate-to-severe).RESULTSThirty-eight of the 52 patients with hPIDs showed common variable immunodeficiency disorders (CVID), while the remaining 14 had CVID-like conditions (i.e., 11 had isolated IgG subclass deficiencies and 3 had selective IgA deficiencies). The prevalence of most HRCT abnormalities was not significantly different between CVID and CVID-like patients (P > 0.05), except for linear and/or irregular opacities (prevalence of 31.6% in the CVID group and 0 in the CVID-like group; P = 0.0427). Airway wall thickening was the most frequent HRCT abnormality found in both CVID and CVID-like patients (71% of cases in both groups). The presence of tree-in-bud abnormalities was an independent predictor of moderate-to-severe obstructive defects at PFTs (Odds Ratio, OR, of 18.75, P < 0.05), while the presence of linear and/or irregular opacities was an independent predictor of restrictive defects at PFTs (OR = 13.00; P < 0.05).CONCLUSIONCVID and CVID-like patients showed similar HRCT findings. Tree-in-bud and linear and/or irregular opacities predicted higher risks of, respectively, obstructive and restrictive defects at PFTs.

Imagerie thoracique : quel futur ?: Thoracic imaging: « bright or no future »?

Short telomeres are frequently identified in patients with idiopathic pulmonary fibrosis (IPF) and its inherited form, familial interstitial pneumonia (FIP). We identified a kindred with FIP with short telomeres who did not carry a mutation in known FIP genes TERT or hTR. We performed targeted sequencing of other telomere-related genes to identify the genetic basis of FIP in this kindred. The proband was a 69 year-old man with dyspnea, restrictive pulmonary function test results, and reticular changes on high-resolution CT scan. An older male sibling had died from IPF. The proband had markedly shortened telomeres in peripheral blood and undetectably short telomeres in alveolar epithelial cells. Polymerase chain reaction-based sequencing of NOP10, TINF2, NHP2, and DKC1 revealed that both affected siblings shared a novel A to G 1213 transition in DKC1 near the hTR binding domain that is predicted to encode a Thr405Ala amino acid substitution. hTR levels were decreased out of proportion to DKC1 expression in the T405A DKC1 proband, suggesting this mutation destabilizes hTR and impairs telomerase function. This DKC1 variant represents the third telomere-related gene identified as a genetic cause of FIP. Further investigation into the mechanism by which dyskerin contributes to the development of lung fibrosis is warranted.

A Novel Dyskerin (DKC1) Mutation Is Associated With Familial Interstitial Pneumonia

Short telomeres are frequently identified in patients with idiopathic pulmonary fibrosis (IPF) and its inherited form, familial interstitial pneumonia (FIP). We identified a kindred with FIP with short telomeres who did not carry a mutation in known FIP genes TERT or hTR . We performed targeted sequencing of other telomere-related genes to identify the genetic basis of FIP in this kindred. The proband was a 69 year-old man with dyspnea, restrictive pulmonary function test results, and reticular changes on high-resolution CT scan. An older male sibling had died from IPF. The proband had markedly shortened telomeres in peripheral blood and undetectably short telomeres in alveolar epithelial cells. Polymerase chain reaction-based sequencing of NOP10 , TINF2 , NHP2 , and DKC1 revealed that both affected siblings shared a novel A to G 1213 transition in DKC1 near the hTR binding domain that is predicted to encode a Thr405Ala amino acid substitution. hTR levels were decreased out of proportion to DKC1 expression in the T405A DKC1 proband, suggesting this mutation destabilizes hTR and impairs telomerase function. This DKC1 variant represents the third telomere-related gene identified as a genetic cause of FIP. Further investigation into the mechanism by which dyskerin contributes to the development of lung fibrosis is warranted.

A 55 years old man with pulmonary alveolar microlithiasis.

Pulmonary alveolar microlithiasis (PAM) is a very rare diffuse chronic lung disease characterized by deposition of small spherules of calcium phosphate within the alveolar cavity. The disease is usually seen from birth up to 40 years of age and is usually diagnosed incidentally during radiography of the chest for other reasons. Most of patients are asymptomatic or having very mild symptoms and the majority of patients either have normal or restrictive pulmonary function test. Clinically, the course of the disease is different; it remains static in few patients or it may progress to pulmonary fibrosis, respiratory failure and cor pulmonale in others. In this case report, we present a 55-year-old man who presented with moderate shortness of breath which has progressed from mild symptoms with in the previous years. His chest high-resolution CT scan showed diffusely scattered, ill-defined little shadowy micronodules which involve the left lung; lingula and left lower lobe in particular. A lung biopsy confirmed the diagnosis of PAM. He was followed up for 1 year with treatment by steroid and alendronate, and no progression was noticed in fact improvement in pulmonary function test noticed. This is the first case report of PAM in Kurdistan.

Pulmonary function and autoantibodies in a long-term follow-up of juvenile dermatomyositis patients

Pulmonary disease is a rare complication in JDM, described in only a few studies. This long-term follow-up study aimed to (i) describe pulmonary involvement in a national cohort of JDM patients estimated by conventional spirometry, (ii) compare pulmonary impairment with overall JDM outcome, and (iii) identify possible associations between pulmonary impairment and myositis-specific autoantibodies (MSAs). Fifty-one JDM patients performed conventional spirometry in a cross-sectional follow-up study. The scores of the Myositis Damage Index (MDI), Myositis Damage by visual analogue scale (MYODAM-VAS) and physician's global damage assessment were used to estimate JDM outcome. ANAs, MSAs and myositis-associated autoantibodies were analysed in all patients. Forty-two patients (82%) (mean follow-up time 14.3 years) had normal lung function. Four patients (8%) were diagnosed with JDM-related restrictive interstitial lung disease. No patients reported pulmonary symptoms. Patients with restrictive pulmonary function had increased long-term damage estimated by MDI (P = 0.008), MYODAM-VAS (P = 0.04), global assessment (P = 0.03) and number of organ systems involved (P = 0.009). We found significant correlation between the restrictive pulmonary function test and damage by the MDI (r = 0.43, P = 0.003), MYODAM-VAS (r = 0.44, P = 0.002), and global damage assessment (r = 0.43, P = 0.003). No association was found between the restrictive pulmonary function test and autoantibodies. In a long-term follow-up study of JDM patients, the majority of patients demonstrated normal lung function. However, restrictive pulmonary impairment was identified in 8% of patients, indicating a need for repetitive pulmonary follow-up in JDM patients. Restrictive pulmonary involvement was associated with increased long-term JDM damage.

The effect of pulse methylprednisolone plus theophylline treatment on clinical, pulmonary and inflammatory markers in relapses of multiple sclerosis.

The aim of this study was to evaluate whether there was a relationship between severity of multiple sclerosis (MS) exacerbation and pulmonary function test (PFT) and to determine the effect of theophylline, which was added to intravenous methylprednisolone, on serum Tumor Necrosis Factor (TNF)-alpha levels and clinical scores in MS relapses. Double blind randomized controlled trial. The baseline Expanded Disability Status Scale (EDSS) score was determined, PFT was performed and blood was taken for analysis of TNF- alpha in patients with MS exacerbation. Patients were randomly divided into two groups; group 1 received intravenous methylprednisolone+IV theophylline and group 2 intravenous methylprednisolone+placebo for 5 days. PFT and EDSS score were repeated and blood was taken for TNF-alpha on the 5(th) and 30(th) days of the treatment. Twenty-four patients (14 female, 10 male) were included in the study. Mean age was 32.6±9, duration of disease was 5.4±4.2 years, number of exacerbations was 5±2. There was a significant correlation between the number of exacerbations and EDSS score (p=0.000, r=1). Restrictive PFT findings were detected in 8 and decrease in carbon monoxide diffusing capacity (DLCO) in 3 cases. In within-group analysis, EDSS score was found to be decreased on day 5 and still low on day 30 in the theophylline group (baseline 3±1.3; 5(th) day 2.4±1.6; 30(th) day 2±1.7). There was no statistically significant difference in the EDSS score of the placebo group (3±1.6; 2.8±1.7; 2.4±1.9 respectively). While serum TNF-alpha level was not changed in the placebo group, there was a non-significant decrease on day 5 and increase on day 30 in the theophylline group. There was no correlation between the clinical parameters, PFT and TNF-alpha level. There was no correlation between severity of MS and PFT findings. It is suggested that theophylline might be effective in MS exacerbations since it caused decreases in clinical scores; studies with longer treatment duration are needed to clarify its possible anti-inflammatory effect.

Examination of late pulmonary toxicity in children treated for malignancies

The present investigation was based on a survey in 2005, in which the authors found pulmonary function abnormalities in survivors of childhood cancer, who were treated with anticancer therapy. The purpose of the present study was to follow-up childhood cancer survivors and detect late pulmonary toxicity. Lung function test was performed with spirometry in 26 survivors participated in this study (10 females and 16 males; mean age, 19.4 years at the time of the second follow-up evaluation). The average time periods from treatment until the first and second follow-up evaluation were 4.5 and 10 years, respectively. The authors found 14 patients with pathological pulmonary function tests results at the time of the first follow-up evaluation, from which 7 patients had obstructive, 5 patients had mixed and 2 patients had restrictive abnormalities. However, there were only 6 patients who had abnormal pulmonary function at the time of the second follow-up evaluation (2 patients with obstructive and 4 patients with restrictive pulmonary function tests (p<0.05). Restrictive pulmonary disorder was detected in only small part of the treated patients. The obstructive pulmonary abnormalities caused by the treatment showed an improving tendency over time.

Assessing Intra-Esophageal Pressure in Patients Presenting with Pulmonary Complaints Using High-Resolution Manometry Presidential Poster

Purpose: The purpose of this study was to determine whether intra-esophageal pressure determined on high-resolution manometry is associated with abnormal pulmonary function. Methods: The Enterprise Data Warehouse (EDW) at Northwestern University identified patients who had undergone HRM and pulmonary function testing within a 6-month period. A retrospective case-control study was performed to evaluate changes in intra-esophageal pressure in patients with underlying pulmonary pathology. A group of 50 asymptomatic volunteers without esophageal dysfunction served as a control group. Intra-esophageal pressure measurements were taken at proximal (3 cm below the UES) and distal (3 cm above the LES) sites, at end-inspiration and end-expiration in the supine position. Participants were then categorized into groups based on pulmonary function testing, using American Thoracic Society guidelines. Statistical analysis using ANOVA evaluated differences in intra-esophageal pressures in patients with normal or restrictive PFTs versus asymptomatic controls. Results: Final analysis included 55 participants with normal PFTs, 55 participants with restrictive PFTs and 50 asymptomatic controls. There was a significant difference in distal esophageal end-expiratory pressure in participants who underwent pulmonary testing (normal and restrictive pulmonary function tests) in comparison to asymptomatic controls (p=7.75 × 10-24). The difference in proximal esophageal end-expiratory pressure in participants with underlying pulmonary pathology was numerically, but not statistically different (p=0.18) (Figure 1). There was a difference between proximal and distal esophageal pressures in all groups in the supine position.Figure 1: Changes in intra-esophageal pressures in individuals with pulmonary disease versus normal controls.Conclusion: Individuals presenting with pulmonary complaints had higher distal esophageal pressures when compared to asymptomatic controls, with the most pronounced difference observed in those with a restrictive pattern during the end-expiratory phase. While the exact mechanism responsible for the pressure differential observed between the proximal and distal esophagus will require further research, this finding suggests the possible influence of poor esophageal emptying or an anatomical or physiologic consequence of the pulmonary disorder as possible causes.

Shrinking lung syndrome in systemic lupus erythematosus patients with dyspnea

Aim of the workTo identify the frequency of shrinking lung syndrome (SLS) in systemic lupus erythematosus (SLE) with dyspnea and study the clinical characteristics and differences in disease activity and damage. Patients and methodsThe study included 47 SLE patients complaining of dyspnea. SLS was considered in those with exertional dyspnea, restrictive pulmonary function tests (PFTs) and elevated copula of the diaphragm. Full history taking, thorough clinical examination, laboratory and relevant radiological investigations were performed for all the patients. Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and Systemic Lupus International Collaborating Clinics (SLICC) indices were compared. High resolution CT chest was performed for patients with radiological findings consistent with SLS. ResultsThe mean age of the patients was 29.43±7.45years, mean disease duration 5.18±3.62years. The SLS was present in 8 patients (17.02%). There was bilateral elevation of the diaphragm copulae in 25% of SLS patients and two had associated basal atelectatic bands. The serum uric acid was significantly higher in those with SLS while the 24h urine protein was significantly lower and C4 normalized. The levels of SLEDAI and SLICC tended to be lower in those with SLS, yet there was no significant difference from those without. The demographic features, clinical and laboratory manifestations, disease activity and damage scores, PFTs and radiological findings of the SLE patients are presented. ConclusionIn SLE patients with dyspnea, SLS should be looked for as it is present in a high proportion of cases.

Chemotherapeutic Agents Increase the Risk for Pulmonary Function Test Abnormalities in Patients With Multiple Myeloma

Case reports of pulmonary toxicity have been published regarding bortezomib, lenalidomide, and thalidomide but there are no published reports looking at the possible long-term pulmonary effects of these medications. This article describes a possible relationship between the administration of bortezomib and thalidomide and the development of pulmonary function test (PFT) abnormalities. It also suggests that routine pulmonary function testing may be required in patients receiving these medications until larger studies can be performed to confirm this observation. Multiple myeloma is a common malignancy accounting for approximately 1% of all malignancies worldwide. Bortezomib, lenalidomide, and thalidomide are immunomodulatory derivatives that are used in the treatment of multiple myeloma (MM). There have been case reports of pulmonary disease associated with these agents, but the effect of these agents on pulmonary function test (PFT) results is unknown. We reviewed the records of 343 patients with MM who underwent PFTs before autologous stem cell transplantation. One hundred nine patients had not received any of the 3 medications, whereas 234 had received 1 or more of these agents. Patients exposed to bortezomib were more likely to have obstructive PFT results (P = .015) when compared with patients not exposed to this medication. Restrictive PFT results were more likely after exposure to thalidomide (P = .017). A logistic regression model was performed and when adjusted for age, sex, Durie-Salmon (DS) stage, body mass index (BMI), time from diagnosis to transplantation in days, and smoking history, the odds of obstruction were 1.96 times higher for patients who received bortezomib. The odds of restriction were 1.97 times higher after exposure to thalidomide. There appears to be a risk of PFT abnormalities developing in patients treated with bortezomib and thalidomide.