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• W4245186928 abstract "In this case, an extensive evaluation of a patient with chest symptoms identified an unusual etiology amenable to a simple treatment. A 40-year-old man presented to our clinic in Bengaluru, India, because of 15 days of fever, breathlessness, and worsening productive cough. He had been treated for repeated episodes of productive cough with intermittent nebulization and expectorants since childhood. Over the 5 years prior to presentation, the cough had become persistent, with expectoration of about 50 mL of mucopurulent sputum per day. Worsening breathlessness during level walking had developed over the past 2 years. The patient also had experienced brief, repeated episodes of diarrhea and colicky abdominal pain since early childhood. These resolved spontaneously and were never associated with fever, blood in stools, or symptoms suggestive of malabsorption. He denied any history of treated tuberculosis, rhinosinusitis, pneumonia, or exanthematous childhood fevers. He had received the bacille Calmette-Guérin tuberculosis vaccine. There was no significant illness in his family, but he was shorter than his siblings. He did not smoke, abuse alcohol or laxatives, or have unprotected sexual intercourse. He was unmarried and a carpenter by occupation. He did not have any pets, and had not noticed any association between his cough and his work. The patient was febrile and normotensive with a respiratory rate of 22 breaths/minute and a pulse of 100 beats/minute. He was only 152 cm in height and malnourished (body mass index, 10.8 Kg/m2; normal, 19-25 Kg/m2). Bilateral pitting pedal edema, grade 2 clubbing, angular chelitis, alopecia, and pallor were present. Breath sounds were reduced bilaterally with coarse, left-sided crackles. Neuromuscular examination showed reduced muscle bulk, proximal weakness, and normal reflexes bilaterally. Spirometry showed evidence of severe airway restriction [forced expiratory volume in 1 sec (FEV1), 0.76 L (28% predicted); forced vital capacity (FVC), 0.86 L (23% predicted); FEV1/FVC ratio, 88%]. Arterial blood gas analysis was normal. Chest radiography (Figure 1) and high-resolution computed tomography (CT) (Figure 2) showed findings suggestive of panlobular emphysema and bronchiectasis.Figure 2High-resolution chest CT scan showing volume loss in the left-lower lung lobe with dilated cystic bronchi reaching the pleura. Varicose bronchiectasis on the right-middle and -lower lobe also is seen. Dense fibrosis in the left-lower lobe, areas of mosaic attenuation, and panlobular emphysema also are observed.View Large Image Figure ViewerDownload Hi-res image Download (PPT) The productive cough, clubbing, coarse crackles, and malnutrition suggested a long-standing suppurative airway disease. The differential diagnosis included bronchiectasis, partially treated lung abscess, chronic empyema with a bronchopleural fistula, and chronic obstructive pulmonary disease (COPD). The onset of symptoms in childhood and the presence of clubbing ruled out COPD and lung abscess, and the lack of pleural symptoms and signs made an empyema unlikely. The CT findings of dilated airways1Cantin L. Bankier A.A. Eisenberg R.L. Bronchiectasis.AJR Am J Roentgenol. 2009; 193: W158-W171Crossref PubMed Scopus (34) Google Scholar confirmed the diagnosis of bronchiectasis. This condition, which is defined by the presence of abnormal, irreversibly dilated medium-sized bronchi,2Pasteur M.C. Bilton D. Hill A.T. British Thoracic Society guideline for non-CF bronchiectasis.Thorax. 2010; 65: i1-i58Crossref PubMed Scopus (489) Google Scholar is declining in incidence in developed countries but remains common in developing countries.3Tsang K.W. Bilton D. Clinical challenges in managing bronchiectasis.Respirology. 2009; 14: 637-650Crossref PubMed Scopus (41) Google Scholar Further testing to determine the etiology of the bronchiectasis showed microcytic, hypochromic anemia (hemoglobin, 9.3 g/dL; mean corpuscular volume, 70 fL; reticulocyte index, 0.8%; platelet count, 4.7×105/μL) with normal leukocyte counts. Serum ferritin and iron were at 15 ng/mL (reference, 30-400 ng/mL) and 30 μg/dL (ref, 65-200 μg/dL), respectively, and total iron binding capacity was 450 μg/dL (ref, 250-350 μg/dL). The fasting blood glucose level was 97 mg/dL, and serum electrolyte and renal function tests were normal. Urine microscopy was negative for albuminuria. Sputum smears were negative for acid-fast bacilli by Zeil-Neelson's staining on 2 occasions, but Pseudomonas aeruginosa was isolated from sputum cultures. The serum creatinine level was 0.3 mg/dL (ref, 0.8-1 mg/dL). Liver function tests showed severe hypoalbuminemia (1.8 g/dL; ref, 4-5 g/dL). Routine examinations of the stool and fecal fat were negative. A d-xylose assay showed evidence of malabsorption (0.48 g excreted in 5 h after a 5-g assay; ref, >0.8 g). A semi-quantitative fecal fat assay was negative (28%). Ultrasound abdominal and colonoscopic exams were normal. An enzyme-linked immunosorbent assay for human immunodeficiency virus (HIV) and an immunofluorescence antinuclear antibody test were negative. Sweat chloride after pilocarpine iontophoresis was 28 mEq/L (abnormal, >70 mEq/L), and serum α1-antitrypsin and serum immunoglobulin (Ig) levels were normal (serum IgG, 1613 mg/dL; ref, 1195-1999 mg/dL; IgE, 30 mg/dL]. Intradermal skin prick testing for Aspergillus fumigatus was negative. The differential diagnosis in patients presenting with gastrointestinal complaints and bronchiectasis includes hypogammaglobulinemia, cystic fibrosis, HIV infection, α1-antitrypsin deficiency, connective tissue disease, and inflammatory bowel disease. The first 4 possibilities were ruled out by the findings of normal serum γ-globulin, sweat chloride, undetectable HIV antibodies, and α1-antitrypsin levels, respectively. The onset of symptoms in childhood and lack of suggestive symptoms argued against a diagnosis of connective tissue disease, and the lack of rectal bleeding and constitutional symptoms argued against inflammatory bowel disease. The patient's severe iron-deficiency anemia, short stature, and malabsorption suggested celiac disease, a diagnosis strongly supported by his extremely high transglutaminase IgA level (292.9 U/mL; ref, <15 U/mL).4Rostom A. Murray J.A. Kagnoff M.F. American Gastroenterological Association (AGA) Institute technical review on the diagnosis and management of celiac disease.Gastroenterology. 2006; 131: 1981-2002Abstract Full Text Full Text PDF PubMed Scopus (585) Google Scholar Because celiac disease is considered rare in southern India and is rarely associated with bronchiectasis, an upper gastrointestinal endoscopy and duodenal biopsy were performed to confirm the diagnosis. Histopathologic analysis of the duodenal biopsy (Figure 3) showed crypt hyperplasia with lamina propria infiltration by lymphocytes; the overlying epithelium and villus architecture was preserved, corresponding to Marsh stage 2 celiac disease.4Rostom A. Murray J.A. Kagnoff M.F. American Gastroenterological Association (AGA) Institute technical review on the diagnosis and management of celiac disease.Gastroenterology. 2006; 131: 1981-2002Abstract Full Text Full Text PDF PubMed Scopus (585) Google Scholar Celiac disease is an immunologic disease with a defined environmental trigger. It is characterized by malabsorption following ingestion of gluten and related proteins (found in wheat, barley, oats, and rye), villus atrophy of small intestinal mucosa, prompt clinical and histologic improvement upon removal of gluten from the diet, and clinical and histologic relapse upon its reintroduction. Cytotoxic and humoral-mediated epithelial toxicity results from the presentation of transglutaminase-deamidated gliadin α-fraction to intestinal mucosal T cells by human leukocyte antigen (HLA)-DQ2. The pulmonary manifestations of celiac disease are varied (Table 1), but because idiopathic pulmonary hemosiderosis (Lane-Hamilton syndrome) is the most common,5Agarwal R. Aggarwal A.N. Gupta D. Lane-Hamilton syndrome: simultaneous occurrence of coeliac disease and idiopathic pulmonary haemosiderosis.Intern Med J. 2007; 37: 65-67Crossref PubMed Scopus (28) Google Scholar all adult patients with this syndrome should be screened for celiac disease. Autopsies and spirometry-based studies have suggested an association between celiac disease and airway disease or obstruction,6Edwards C. Williams A. Asquith P. Bronchopulmonary disease in coeliac patients.J Clin Pathol. 1985; 38: 361-367Crossref PubMed Scopus (16) Google Scholar, 7Tarlo S.M. Broder I. Prokipchuk E.J. Peress L. Mintz S. Association between celiac disease and lung disease.Chest. 1981; 80: 715-718Crossref PubMed Scopus (20) Google Scholar but to the best of our knowledge, only one other case of high-resolution CT-proven bronchiectasis related to celiac disease has been reported.8Mahadeva R. Flower C. Shneerson J. Bronchiectasis in association with coeliac disease.Thorax. 1998; 53: 527-529Crossref PubMed Scopus (15) Google Scholar Bronchiectasis and celiac disease might be linked through exposure to a common antigen, immune complex absorption through deranged gastrointestinal mucosa, or common, immunologically-mediated, epithelial damage. In this regard, these manifestations are analogous to pulmonary involvement in inflammatory bowel disease.Table 1Recommended Initial Evaluation in All Newly Diagnosed Adult Patients with Bronchiectasis and Unclear Etiology(Adapted from BTS Guidelines1Cantin L. Bankier A.A. Eisenberg R.L. Bronchiectasis.AJR Am J Roentgenol. 2009; 193: W158-W171Crossref PubMed Scopus (34) Google Scholar)RecommendationDisease SuspectedStrength of RecommendationHistory and examination for prior lower respiratory disease, pertussis and tuberculosis and temporal relationship with symptoms in all patientsPost-necrotizing pneumonia, tuberculosis[C]Serum immunoglobulins (IgG, IgA, IgM) and serum electrophoresis in all patientsHypogammaglobulinemia[A]Serum IgE, Aspergillus fumigatus RAST/CAP in all patientsAllergic Bronchopulmonary aspergillosis[C]Screening by 2 sweat chloride and mutation analysis in all adults up to age of 40 yearsCystic fibrosis[D]In adults, saccharin and/or Exhaled FeNO only if infertility or repeated upper respiratory abnormalitiesImmotile cilia syndrome[D]Sputum mycobacterial cultureTuberculosis and Non-tubercular mycobacteria[D]Rheumatoid arthritis (RA) by history in all patientsR.A-related bronchiectasis[C]Congenital causes should be considered in all patients (assessment is by HRCT?)Tracheobronchomegaly, William-Campbell syndrome, Sequestration and CCAM[D]Gastric aspiration should be considered in all patientsAspiration-related[D]α1-Antitrypsin levels only if associated basal-predominant emphysemaα1-Antitrypsin deficiency[D]Bronchoscopy only if localized bronchiectasisForeign body, localized tumor[D]Diffuse Pan-bronchiolitis (DPB)-East Asia (HRCT?)DPB[D]History and examination for evidence of yellow nail syndrome in all patientsYellow nail syndrome[D]IgG subtyping, response to vaccination and cell-mediated immune responsesUnclear etiology after initial investigations are negative[D]Key to evidence statements and grades of recommendations in BTS guidelines (1)1++High quality meta-analyses, systematic reviews of randomized controlled trials (RCTs) or RCTs with a very low risk of bias1+Well-conducted meta-analyses, systematic reviews or RCTs with a low risk of bias1−Meta-analyses, systematic reviews or RCTs with a high risk of bias2++High quality systematic reviews of case-control or cohort studies; High quality case-control or cohort studies with a very low risk of confounding or bias and a high probability that the relationship is causal2+Well-conducted case-control or cohort studies with a low risk of confounding or bias and a moderate probability that the relationship is causal2−Case-control or cohort studies with a high risk of confounding or bias and a significant risk that the relationship is not causal3Non-analytic studies (eg, case reports, case series)4Expert opinionAAt least one meta-analysis, systematic review or RCT rated as 1++ and directly applicable to the target population; or A body of evidence consisting principally of studies rated as 1+, directly applicable to the target population, and demonstrating overall consistency of resultsBA body of evidence including studies rated as 2++, directly applicable to the target population, and demonstrating overall consistency of results; or Extrapolated evidence from studies rated as 1++ or 1+CA body of evidence including studies rated as 2+, directly applicable to the target population and demonstrating overall consistency of results; or Extrapolated evidence from studies rated as 2++DEvidence level 3 or 4; or Extrapolated evidence from studies rated as 2+ Open table in a new tab Celiac disease is considered rare in southern India, where the predominant food grain is rice; however, it is prevalent in the Bengal and Punjab provinces of northwest India, where the predominant food grain is wheat. Given the shared ethnicity throughout India, celiac disease is probably under-recognized in southern India, although variations in HLA haplotype, the timing of gluten introduction into diet, and the gliadin concentration of infant formula may contribute to regional differences in its prevalence.9Malekzadeh R. Sachdev A. Ali A.F. Coeliac disease in developing countries: Middle East, India and North Africa.Best Pract Res Clin Gastroenterol. 2005; 19: 351-358Abstract Full Text Full Text PDF PubMed Scopus (63) Google Scholar Procedures for the diagnosis, investigation, and management of bronchiectasis are largely empirical and vary widely. In the developing world, where post-infectious causes of bronchiectasis are rampant, genetic and serologic assays are often limited in availability and expensive, and most bronchiectasis cases (82%) are idiopathic,3Tsang K.W. Bilton D. Clinical challenges in managing bronchiectasis.Respirology. 2009; 14: 637-650Crossref PubMed Scopus (41) Google Scholar the value of etiological evaluation can be unclear. On the other hand, an extensive evaluation of adult bronchiectasis in the developed world found an etiological cause in 47% (70/150) of the patients, leading to a treatment change and improvement in 15% (22/150) of them,10Pasteur M.C. Helliwell S.M. Houghton S.J. Webb S.C. Foweraker J.E. Coulden R.A. et al.An investigation into causative factors in patients with bronchiectasis.Am J Respir Crit Care Med. 2000; 162: 1277-1284Crossref PubMed Scopus (517) Google Scholar suggesting that etiology should be determined wherever possible. A ranked summary of the recommended tests for the initial evaluation of patients with bronchiectasis according to the British Thoracic Society guidelines2Pasteur M.C. Bilton D. Hill A.T. British Thoracic Society guideline for non-CF bronchiectasis.Thorax. 2010; 65: i1-i58Crossref PubMed Scopus (489) Google Scholar is shown in Table 2.Table 2Spectrum of Pulmonary Manifestations of Celiac DiseaseIdiopathic pulmonary hemosiderosis (Lane-Hamilton syndrome)Association with hypersensitivity pneumonitisSarcoidosisLung abscessesAsthmaTuberculosisNon-tubercular mycobacterial infectionsBronchiectasisAsymptomatic bronchoalveolitis Open table in a new tab Gastrointestinal complications of bronchiectasis may include reactive amyloidosis, malnutrition, fatty hepatomegaly, or gastrointestinal tuberculosis. Splenic atrophy and IgA deficiency are associated with bronchiectasis in celiac disease, but a review of our patient's evaluative findings ruled out these conditions. The patient was placed on a gluten-free diet and prescribed inhaled budesonide (200 μg daily). After 1 year on this regimen, he had gained a substantial amount of weight (10 kg), his gastrointestinal complaints had not recurred, and the severity of his cough had decreased. A systematic search of PUBMED was performed by 2 of the authors (RS and UD) using the terms Non-tropical sprue or celiac and Lung or pulmonary. Our search yielded 223 references. The abstracts and full text, where available, of all the articles were searched for pulmonary associations of celiac disease. We also supplemented this search by searching all the references of the abstracted references, searching 2 pulmonology textbooks (Fishman's and Murray & Nadel) and our personal databases." @default.
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