Download PDF
Semin Respir Crit Care Med 2014; 35(02): 274-282
DOI: 10.1055/s-0034-1371538
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Supportive Care for Patients with Pulmonary Complications of Connective Tissue Disease

Mridu Gulati
1   Section of Pulmonary, Critical Care and Sleep Medicine, Department of Internal Medicine, Yale University School of Medicine, New Haven, Connecticut
,
Danielle Antin-Ozerkis
1   Section of Pulmonary, Critical Care and Sleep Medicine, Department of Internal Medicine, Yale University School of Medicine, New Haven, Connecticut
› Author Affiliations
Further Information

Publication History

Publication Date:
25 March 2014 (online)

    Permissions and Reprints

Abstract

Patients with connective tissue disease often suffer from pulmonary complications, including interstitial lung disease and pulmonary hypertension. Supportive care for these patients aims to relieve symptoms and improve activity level and quality of life. A holistic approach to the management of patients with advanced connective tissue disease–associated pulmonary disorders includes a full assessment of patient symptoms as well as a careful search for side effects of treatment and treatable comorbidities. This article addresses supportive measures such as supplemental oxygen and pulmonary rehabilitation. Issues related to quality of life, sleep disturbances, and identification of mood disorders are discussed. In addition, we review significant comorbidities, including cardiovascular disease, glucocorticoid-induced osteoporosis, and gastroesophageal reflux disease. Essential facets of advanced lung disease, including mechanical ventilation, lung transplantation, end-of-life care, and hospice, are covered.

Keywords

connective tissue disease - interstitial lung disease - pulmonary hypertension - supportive care - oxygen - pulmonary rehabilitation
 

  • References

  • 1 Nocturnal Oxygen Therapy Trial Group. Continuous or nocturnal oxygen therapy in hypoxemic chronic obstructive lung disease: a clinical trial. Ann Intern Med 1980; 93 (3) 391-398
    CrossrefPubMedGoogle Scholar
  • 2 Report of the Medical Research Council Working Party. Long term domiciliary oxygen therapy in chronic hypoxic cor pulmonale complicating chronic bronchitis and emphysema. Lancet 1981; 1 (8222) 681-686
    PubMedGoogle Scholar
  • 3 Timms RM, Khaja FU, Williams GW. Hemodynamic response to oxygen therapy in chronic obstructive pulmonary disease. Ann Intern Med 1985; 102 (1) 29-36
    CrossrefPubMedGoogle Scholar
  • 4 Selinger SR, Kennedy TP, Buescher P , et al. Effects of removing oxygen from patients with chronic obstructive pulmonary disease. Am Rev Respir Dis 1987; 136 (1) 85-91
    CrossrefPubMedGoogle Scholar
  • 5 Criner GJ. Ambulatory home oxygen: what is the evidence for benefit, and who does it help?. Respir Care 2013; 58 (1) 48-64
    CrossrefPubMedGoogle Scholar
  • 6 Bjoraker JA, Ryu JH, Edwin MK , et al. Prognostic significance of histopathologic subsets in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 1998; 157 (1) 199-203
    CrossrefPubMedGoogle Scholar
  • 7 Chetta A, Aiello M, Foresi A , et al. Relationship between outcome measures of six-minute walk test and baseline lung function in patients with interstitial lung disease. Sarcoidosis Vasc Diffuse Lung Dis 2001; 18 (2) 170-175
    PubMedGoogle Scholar
  • 8 Knower MT, Dunagan DP, Adair NE, Chin Jr R. Baseline oxygen saturation predicts exercise desaturation below prescription threshold in patients with chronic obstructive pulmonary disease. Arch Intern Med 2001; 161 (5) 732-736
    CrossrefPubMedGoogle Scholar
  • 9 O'Donnell DE, Fitzpatrick MF. Physiology of interstitial lung disease. In: Schwarz MI, King TE, , eds. Interstitial Lung Disease. Ontario, Canada: BC Decker; 2003
    Google Scholar
  • 10 Hughes JM, Lockwood DN, Jones HA, Clark RJ. DLCO/Q and diffusion limitation at rest and on exercise in patients with interstitial fibrosis. Respir Physiol 1991; 83 (2) 155-166
    CrossrefPubMedGoogle Scholar
  • 11 Paolillo S, Farina S, Bussotti M , et al. Exercise testing in the clinical management of patients affected by pulmonary arterial hypertension. Eur J Prev Cardiol 2012; 19 (5) 960-971
    CrossrefPubMedGoogle Scholar
  • 12 Bradley B, Branley HM, Egan JJ , et al; British Thoracic Society Interstitial Lung Disease Guideline Group, British Thoracic Society Standards of Care Committee; Thoracic Society of Australia; New Zealand Thoracic Society; Irish Thoracic Society. Interstitial lung disease guideline: the British Thoracic Society in collaboration with the Thoracic Society of Australia and New Zealand and the Irish Thoracic Society. Thorax 2008; 63 (Suppl. 05) v1-v58
    CrossrefPubMedGoogle Scholar
  • 13 Harris-Eze AO, Sridhar G, Clemens RE, Gallagher CG, Marciniuk DD. Oxygen improves maximal exercise performance in interstitial lung disease. Am J Respir Crit Care Med 1994; 150 (6, Pt 1) 1616-1622
    CrossrefPubMedGoogle Scholar
  • 14 Clark M, Cooper B, Singh S, Cooper M, Carr A, Hubbard R. A survey of nocturnal hypoxaemia and health related quality of life in patients with cryptogenic fibrosing alveolitis. Thorax 2001; 56 (6) 482-486
    CrossrefPubMedGoogle Scholar
  • 15 Leggett RJ, Flenley DC. Portable oxygen and exercise tolerance in patients with chronic hypoxic cor pulmonale. BMJ 1977; 2 (6079) 84-86
    CrossrefPubMedGoogle Scholar
  • 16 Nici L, Donner C, Wouters E , et al; ATS/ERS Pulmonary Rehabilitation Writing Committee. American Thoracic Society/European Respiratory Society statement on pulmonary rehabilitation. Am J Respir Crit Care Med 2006; 173 (12) 1390-1413
    CrossrefPubMedGoogle Scholar
  • 17 Holland AE, Hill CJ, Conron M, Munro P, McDonald CF. Short term improvement in exercise capacity and symptoms following exercise training in interstitial lung disease. Thorax 2008; 63 (6) 549-554
    CrossrefPubMedGoogle Scholar
  • 18 Griffiths TL, Burr ML, Campbell IA , et al. Results at 1 year of outpatient multidisciplinary pulmonary rehabilitation: a randomised controlled trial. Lancet 2000; 355 (9201) 362-368
    CrossrefPubMedGoogle Scholar
  • 19 Egan JJ. Follow-up and nonpharmacological management of the idiopathic pulmonary fibrosis patient. Eur Respir Rev 2011; 20 (120) 114-117
    CrossrefPubMedGoogle Scholar
  • 20 Laviolette L, Bourbeau J, Bernard S , et al. Assessing the impact of pulmonary rehabilitation on functional status in COPD. Thorax 2008; 63 (2) 115-121
    PubMedGoogle Scholar
  • 21 Holland A, Hill C. Physical training for interstitial lung disease. Cochrane Database Syst Rev 2008; (4) CD006322
    PubMedGoogle Scholar
  • 22 Swigris JJ, Brown KK, Make BJ, Wamboldt FS. Pulmonary rehabilitation in idiopathic pulmonary fibrosis: a call for continued investigation. Respir Med 2008; 102 (12) 1675-1680
    CrossrefPubMedGoogle Scholar
  • 23 Garvey C. Interstitial lung disease and pulmonary rehabilitation. J Cardiopulm Rehabil Prev 2010; 30 (3) 141-146
    CrossrefPubMedGoogle Scholar
  • 24 Salhi B, Troosters T, Behaegel M, Joos G, Derom E. Effects of pulmonary rehabilitation in patients with restrictive lung diseases. Chest 2010; 137 (2) 273-279
    CrossrefPubMedGoogle Scholar
  • 25 Ryerson CJ, Garvey C, Collard HR. Pulmonary rehabilitation for interstitial lung disease. Chest 2010; 138 (1) 240-241 , author reply 241–242
    CrossrefPubMedGoogle Scholar
  • 26 Ferreira A, Garvey C, Connors GL , et al. Pulmonary rehabilitation in interstitial lung disease: benefits and predictors of response. Chest 2009; 135 (2) 442-447
    CrossrefPubMedGoogle Scholar
  • 27 Singer HK, Ruchinskas RA, Riley KC, Broshek DK, Barth JT. The psychological impact of end-stage lung disease. Chest 2001; 120 (4) 1246-1252
    CrossrefPubMedGoogle Scholar
  • 28 Munsterman T, Takken T, Wittink H. Are persons with rheumatoid arthritis deconditioned? A review of physical activity and aerobic capacity. BMC Musculoskelet Disord 2012; 13: 202
    CrossrefPubMedGoogle Scholar
  • 29 Iversen MD, Brandenstein JS. Do dynamic strengthening and aerobic capacity exercises reduce pain and improve functional outcomes and strength in people with established rheumatoid arthritis?. Phys Ther 2012; 92 (10) 1251-1257
    CrossrefPubMedGoogle Scholar
  • 30 Cairns AP, McVeigh JG. A systematic review of the effects of dynamic exercise in rheumatoid arthritis. Rheumatol Int 2009; 30 (2) 147-158
    CrossrefPubMedGoogle Scholar
  • 31 Alexanderson H, Lundberg IE. Exercise as a therapeutic modality in patients with idiopathic inflammatory myopathies. Curr Opin Rheumatol 2012; 24 (2) 201-207
    CrossrefPubMedGoogle Scholar
  • 32 Perandini LA, de Sá-Pinto AL, Roschel H , et al. Exercise as a therapeutic tool to counteract inflammation and clinical symptoms in autoimmune rheumatic diseases. Autoimmun Rev 2012; 12 (2) 218-224
    CrossrefPubMedGoogle Scholar
  • 33 Carvalho MR, Sato EI, Tebexreni AS, Heidecher RT, Schenkman S, Neto TL. Effects of supervised cardiovascular training program on exercise tolerance, aerobic capacity, and quality of life in patients with systemic lupus erythematosus. Arthritis Rheum 2005; 53 (6) 838-844
    CrossrefPubMedGoogle Scholar
  • 34 Oliveira NC, dos Santos Sabbag LM, de Sá Pinto AL, Borges CL, Lima FR. Aerobic exercise is safe and effective in systemic sclerosis. Int J Sports Med 2009; 30 (10) 728-732
    Article in Thieme ConnectPubMedGoogle Scholar
  • 35 Mahler DA. Opioids for refractory dyspnea. Expert Rev Respir Med 2013; 7 (2) 123-134 , quiz 135
    CrossrefPubMedGoogle Scholar
  • 36 O'Donnell DE, Ora J, Webb KA, Laveneziana P, Jensen D. Mechanisms of activity-related dyspnea in pulmonary diseases. Respir Physiol Neurobiol 2009; 167 (1) 116-132
    CrossrefPubMedGoogle Scholar
  • 37 Mahler DA, Selecky PA, Harrod CG , et al. American College of Chest Physicians consensus statement on the management of dyspnea in patients with advanced lung or heart disease. Chest 2010; 137 (3) 674-691
    CrossrefPubMedGoogle Scholar
  • 38 Gilbert CR, Smith CM. Advanced lung disease: quality of life and role of palliative care. Mt Sinai J Med 2009; 76 (1) 63-70
    CrossrefPubMedGoogle Scholar
  • 39 Bajwah S, Higginson IJ, Ross JR , et al. Specialist palliative care is more than drugs: a retrospective study of ILD patients. Lung 2012; 190 (2) 215-220
    CrossrefPubMedGoogle Scholar
  • 40 Bajwah S, Higginson IJ, Ross JR , et al. The palliative care needs for fibrotic interstitial lung disease: a qualitative study of patients, informal caregivers and health professionals. Palliat Med 2013; 27 (9) 869-876
    CrossrefPubMedGoogle Scholar
  • 41 Bajwah S, Ross JR, Peacock JL , et al. Interventions to improve symptoms and quality of life of patients with fibrotic interstitial lung disease: a systematic review of the literature. Thorax 2013; 68 (9) 867-879
    CrossrefPubMedGoogle Scholar
  • 42 Light RW, Muro JR, Sato RI, Stansbury DW, Fischer CE, Brown SE. Effects of oral morphine on breathlessness and exercise tolerance in patients with chronic obstructive pulmonary disease. Am Rev Respir Dis 1989; 139 (1) 126-133
    CrossrefPubMedGoogle Scholar
  • 43 Wee B, Browning J, Adams A , et al. Management of chronic cough in patients receiving palliative care: review of evidence and recommendations by a task group of the Association for Palliative Medicine of Great Britain and Ireland. Palliat Med 2012; 26 (6) 780-787
    CrossrefPubMedGoogle Scholar
  • 44 Horton MR, Santopietro V, Mathew L , et al. Thalidomide for the treatment of cough in idiopathic pulmonary fibrosis: a randomized trial. Ann Intern Med 2012; 157 (6) 398-406
    CrossrefPubMedGoogle Scholar
  • 45 Pincus T, Griffith J, Pearce S, Isenberg D. Prevalence of self-reported depression in patients with rheumatoid arthritis. Br J Rheumatol 1996; 35 (9) 879-883
    CrossrefPubMedGoogle Scholar
  • 46 Zheng J, Umikawa M, Cui C , et al. Inhibitory receptors bind ANGPTLs and support blood stem cells and leukaemia development. Nature 2012; 485 (7400) 656-660
    CrossrefPubMedGoogle Scholar
  • 47 Odegård S, Finset A, Mowinckel P, Kvien TK, Uhlig T. Pain and psychological health status over a 10-year period in patients with recent onset rheumatoid arthritis. Ann Rheum Dis 2007; 66 (9) 1195-1201
    CrossrefPubMedGoogle Scholar
  • 48 Jewett LR, Razykov I, Hudson M, Baron M, Thombs BD ; Canadian Scleroderma Research Group. Prevalence of current, 12-month and lifetime major depressive disorder among patients with systemic sclerosis. Rheumatology (Oxford) 2013; 52 (4) 669-675
    CrossrefPubMedGoogle Scholar
  • 49 Akhtar AA, Ali MA, Smith RP. Depression in patients with idiopathic pulmonary fibrosis. Chron Respir Dis 2013; 10 (3) 127-133
    CrossrefPubMedGoogle Scholar
  • 50 De Vries J, Kessels BL, Drent M. Quality of life of idiopathic pulmonary fibrosis patients. Eur Respir J 2001; 17 (5) 954-961
    CrossrefPubMedGoogle Scholar
  • 51 Lindell KO, Olshansky E, Song MK , et al. Impact of a disease-management program on symptom burden and health-related quality of life in patients with idiopathic pulmonary fibrosis and their care partners. Heart Lung 2010; 39 (4) 304-313
    CrossrefPubMedGoogle Scholar
  • 52 Ho RC, Fu EH, Chua AN, Cheak AA, Mak A. Clinical and psychosocial factors associated with depression and anxiety in Singaporean patients with rheumatoid arthritis. Int J Rheum Dis 2011; 14 (1) 37-47
    CrossrefPubMedGoogle Scholar
  • 53 Kekow J, Moots R, Khandker R, Melin J, Freundlich B, Singh A. Improvements in patient-reported outcomes, symptoms of depression and anxiety, and their association with clinical remission among patients with moderate-to-severe active early rheumatoid arthritis. Rheumatology (Oxford) 2011; 50 (2) 401-409
    CrossrefPubMedGoogle Scholar
  • 54 Overman CL, Bossema ER, van Middendorp H , et al. The prospective association between psychological distress and disease activity in rheumatoid arthritis: a multilevel regression analysis. Ann Rheum Dis 2012; 71 (2) 192-197
    PubMedGoogle Scholar
  • 55 Abad VC, Sarinas PS, Guilleminault C. Sleep and rheumatologic disorders. Sleep Med Rev 2008; 12 (3) 211-228
    CrossrefPubMedGoogle Scholar
  • 56 Mermigkis C, Stagaki E, Amfilochiou A , et al. Sleep quality and associated daytime consequences in patients with idiopathic pulmonary fibrosis. Medical principles and practice: international journal of the Kuwait University. Health Science Centre 2009; 18: 10-15
    PubMedGoogle Scholar
  • 57 Hirsch M, Carlander B, Vergé M , et al. Objective and subjective sleep disturbances in patients with rheumatoid arthritis. A reappraisal. Arthritis Rheum 1994; 37 (1) 41-49
    CrossrefPubMedGoogle Scholar
  • 58 Mahowald MW, Mahowald ML, Bundlie SR, Ytterberg SR. Sleep fragmentation in rheumatoid arthritis. Arthritis Rheum 1989; 32 (8) 974-983
    CrossrefPubMedGoogle Scholar
  • 59 Prado GF, Allen RP, Trevisani VM, Toscano VG, Earley CJ. Sleep disruption in systemic sclerosis (scleroderma) patients: clinical and polysomnographic findings. Sleep Med 2002; 3 (4) 341-345
    CrossrefPubMedGoogle Scholar
  • 60 Drossaers-Bakker KW, Hamburger HL, Bongartz EB, Dijkmans BA, Van Soesbergen RM. Sleep apnoea caused by rheumatoid arthritis. Br J Rheumatol 1998; 37 (8) 889-894
    CrossrefPubMedGoogle Scholar
  • 61 Lancaster LH, Mason WR, Parnell JA , et al. Obstructive sleep apnea is common in idiopathic pulmonary fibrosis. Chest 2009; 136 (3) 772-778
    CrossrefPubMedGoogle Scholar
  • 62 Mermigkis C, Stagaki E, Tryfon S , et al. How common is sleep-disordered breathing in patients with idiopathic pulmonary fibrosis?. Sleep Breath 2010; 14 (4) 387-390
    CrossrefPubMedGoogle Scholar
  • 63 Rasche K, Orth M. Sleep and breathing in idiopathic pulmonary fibrosis. J Physiol Pharmacol 2009; 60 (Suppl. 05) 13-14
    PubMedGoogle Scholar
  • 64 Selim B, Won C, Yaggi HK. Cardiovascular consequences of sleep apnea. Clin Chest Med 2010; 31 (2) 203-220
    CrossrefPubMedGoogle Scholar
  • 65 Kalluri M, Oddis CV. Pulmonary manifestations of the idiopathic inflammatory myopathies. Clin Chest Med 2010; 31 (3) 501-512
    CrossrefPubMedGoogle Scholar
  • 66 Arend WP, Gabay C. Cytokines in the rheumatic diseases. Rheum Dis Clin North Am 2004; 30 (1) 41-67 , v–vi
    CrossrefPubMedGoogle Scholar
  • 67 Vgontzas AN, Zoumakis E, Lin HM, Bixler EO, Trakada G, Chrousos GP. Marked decrease in sleepiness in patients with sleep apnea by etanercept, a tumor necrosis factor-alpha antagonist. J Clin Endocrinol Metab 2004; 89 (9) 4409-4413
    CrossrefPubMedGoogle Scholar
  • 68 Krishnan V, McCormack MC, Mathai SC , et al. Sleep quality and health-related quality of life in idiopathic pulmonary fibrosis. Chest 2008; 134 (4) 693-698
    CrossrefPubMedGoogle Scholar
  • 69 Mermigkis C, Bouloukaki I, Antoniou KM , et al. CPAP therapy in patients with idiopathic pulmonary fibrosis and obstructive sleep apnea: does it offer a better quality of life and sleep?. Sleep Breath 2013; 17 (4) 1137-1143
    CrossrefPubMedGoogle Scholar
  • 70 Dumitrascu R, Tiede H, Eckermann J , et al. Sleep apnea in precapillary pulmonary hypertension. Sleep Med 2013; 14 (3) 247-251
    CrossrefPubMedGoogle Scholar
  • 71 Ponnuswamy A, Manikandan R, Sabetpour A, Keeping IM, Finnerty JP. Association between ischaemic heart disease and interstitial lung disease: a case-control study. Respir Med 2009; 103 (4) 503-507
    CrossrefPubMedGoogle Scholar
  • 72 Nathan SD, Basavaraj A, Reichner C , et al. Prevalence and impact of coronary artery disease in idiopathic pulmonary fibrosis. Respir Med 2010; 104 (7) 1035-1041
    CrossrefPubMedGoogle Scholar
  • 73 Villa-Forte A, Mandell BF. Cardiovascular disorders and rheumatic disease [in Spanish]. Rev Esp Cardiol 2011; 64 (9) 809-817
    CrossrefPubMedGoogle Scholar
  • 74 Adzić TN, Pesut DP, Nagorni-Obradović LM, Stojsić JM, Vasiljević MD, Bouros D. Clinical features of lung cancer in patients with connective tissue diseases: a 10-year hospital based study. Respir Med 2008; 102 (4) 620-624
    CrossrefPubMedGoogle Scholar
  • 75 Khurana R, Wolf R, Berney S, Caldito G, Hayat S, Berney SM. Risk of development of lung cancer is increased in patients with rheumatoid arthritis: a large case control study in US veterans. J Rheumatol 2008; 35 (9) 1704-1708
    PubMedGoogle Scholar
  • 76 Sode BF, Dahl M, Nielsen SF, Nordestgaard BG. Venous thromboembolism and risk of idiopathic interstitial pneumonia: a nationwide study. Am J Respir Crit Care Med 2010; 181 (10) 1085-1092
    CrossrefPubMedGoogle Scholar
  • 77 Petri M. Update on anti-phospholipid antibodies in SLE: the Hopkins' Lupus Cohort. Lupus 2010; 19 (4) 419-423
    CrossrefPubMedGoogle Scholar
  • 78 Patel NM, Lederer DJ, Borczuk AC, Kawut SM. Pulmonary hypertension in idiopathic pulmonary fibrosis. Chest 2007; 132 (3) 998-1006
    CrossrefPubMedGoogle Scholar
  • 79 Goldberg A. Pulmonary arterial hypertension in connective tissue diseases. Cardiol Rev 2010; 18 (2) 85-88
    CrossrefPubMedGoogle Scholar
  • 80 Corte TJ, Wort SJ, Wells AU. Pulmonary hypertension in idiopathic pulmonary fibrosis: a review. Sarcoidosis Vasc Diffuse Lung Dis 2009; 26 (1) 7-19
    PubMedGoogle Scholar
  • 81 Seguro LP, Rosario C, Shoenfeld Y. Long-term complications of past glucocorticoid use. Autoimmun Rev 2013; 12 (5) 629-632
    CrossrefPubMedGoogle Scholar
  • 82 Wei L, MacDonald TM, Walker BR. Taking glucocorticoids by prescription is associated with subsequent cardiovascular disease. Ann Intern Med 2004; 141 (10) 764-770
    CrossrefPubMedGoogle Scholar
  • 83 Compston J. Clinical question: What is the best approach to managing glucocorticoid-induced osteoporosis?. Clin Endocrinol (Oxf) 2011; 74 (5) 547-550
    CrossrefPubMedGoogle Scholar
  • 84 Compston J. Management of glucocorticoid-induced osteoporosis. Nat Rev Rheumatol 2010; 6 (2) 82-88
    CrossrefPubMedGoogle Scholar
  • 85 Kanis JA, Johansson H, Oden A , et al. A family history of fracture and fracture risk: a meta-analysis. Bone 2004; 35 (5) 1029-1037
    CrossrefPubMedGoogle Scholar
  • 86 Maricic M. Update on glucocorticoid-induced osteoporosis. Rheum Dis Clin North Am 2011; 37 (3) 415-431 , vi
    CrossrefPubMedGoogle Scholar
  • 87 Pereira RM, Carvalho JF, Paula AP , et al; Committee for Osteoporosis and Bone Metabolic Disorders of the Brazilian Society of Rheumatology; Brazilian Medical Association; Brazilian Association of Physical Medicine and Rehabilitation. Guidelines for the prevention and treatment of glucocorticoid-induced osteoporosis. Rev Bras Reumatol 2012; 52 (4) 580-593
    CrossrefPubMedGoogle Scholar
  • 88 Lekamwasam S, Adachi JD, Agnusdei D , et al; Joint IOF-ECTS GIO Guidelines Working Group. A framework for the development of guidelines for the management of glucocorticoid-induced osteoporosis. Osteoporos Int 2012; 23 (9) 2257-2276
    CrossrefPubMedGoogle Scholar
  • 89 Grossman JM, Gordon R, Ranganath VK , et al. American College of Rheumatology 2010 recommendations for the prevention and treatment of glucocorticoid-induced osteoporosis. Arthritis Care Res (Hoboken) 2010; 62 (11) 1515-1526
    CrossrefPubMedGoogle Scholar
  • 90 Tobin RW, Pope II CE, Pellegrini CA, Emond MJ, Sillery J, Raghu G. Increased prevalence of gastroesophageal reflux in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 1998; 158 (6) 1804-1808
    CrossrefPubMedGoogle Scholar
  • 91 Raghu G, Freudenberger TD, Yang S , et al. High prevalence of abnormal acid gastro-oesophageal reflux in idiopathic pulmonary fibrosis. Eur Respir J 2006; 27 (1) 136-142
    CrossrefPubMedGoogle Scholar
  • 92 Soares RV, Forsythe A, Hogarth K, Sweiss NJ, Noth I, Patti MG. Interstitial lung disease and gastroesophageal reflux disease: key role of esophageal function tests in the diagnosis and treatment. Arq Gastroenterol 2011; 48 (2) 91-97
    CrossrefPubMedGoogle Scholar
  • 93 Christmann RB, Wells AU, Capelozzi VL, Silver RM. Gastroesophageal reflux incites interstitial lung disease in systemic sclerosis: clinical, radiologic, histopathologic, and treatment evidence. Semin Arthritis Rheum 2010; 40 (3) 241-249
    CrossrefPubMedGoogle Scholar
  • 94 de Souza RB, Borges CT, Capelozzi VL , et al. Centrilobular fibrosis: an underrecognized pattern in systemic sclerosis. Respiration 2009; 77 (4) 389-397
    CrossrefPubMedGoogle Scholar
  • 95 Raghu G, Yang ST, Spada C, Hayes J, Pellegrini CA. Sole treatment of acid gastroesophageal reflux in idiopathic pulmonary fibrosis: a case series. Chest 2006; 129 (3) 794-800
    CrossrefPubMedGoogle Scholar
  • 96 Lee JS, Ryu JH, Elicker BM , et al. Gastroesophageal reflux therapy is associated with longer survival in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2011; 184 (12) 1390-1394
    CrossrefPubMedGoogle Scholar
  • 97 Linden PA, Gilbert RJ, Yeap BY , et al. Laparoscopic fundoplication in patients with end-stage lung disease awaiting transplantation. J Thorac Cardiovasc Surg 2006; 131 (2) 438-446
    CrossrefPubMedGoogle Scholar
  • 98 Forbes A, Marie I. Gastrointestinal complications: the most frequent internal complications of systemic sclerosis. Rheumatology (Oxford) 2009; 48 (Suppl. 03) iii36-iii39
    PubMedGoogle Scholar
  • 99 Marie I, Dominique S, Levesque H , et al. Esophageal involvement and pulmonary manifestations in systemic sclerosis. Arthritis Rheum 2001; 45 (4) 346-354
    CrossrefPubMedGoogle Scholar
  • 100 Savarino E, Bazzica M, Zentilin P , et al. Gastroesophageal reflux and pulmonary fibrosis in scleroderma: a study using pH-impedance monitoring. Am J Respir Crit Care Med 2009; 179 (5) 408-413
    CrossrefPubMedGoogle Scholar
  • 101 Marie I, Levesque H, Ducrotté P , et al. Gastric involvement in systemic sclerosis: a prospective study. Am J Gastroenterol 2001; 96 (1) 77-83
    CrossrefPubMedGoogle Scholar
  • 102 Fagundes MN, Caleiro MT, Navarro-Rodriguez T , et al. Esophageal involvement and interstitial lung disease in mixed connective tissue disease. Respir Med 2009; 103 (6) 854-860
    CrossrefPubMedGoogle Scholar
  • 103 Yusen RD, Christie JD, Edwards LB , et al; International Society for Heart and Lung Transplantation. The Registry of the International Society for Heart and Lung Transplantation: thirtieth adult lung and heart-lung transplant report—2013; focus theme: age. J Heart Lung Transplant 2013; 32 (10) 965-978
    CrossrefPubMedGoogle Scholar
  • 104 Christie JD, Edwards LB, Kucheryavaya AY , et al. The Registry of the International Society for Heart and Lung Transplantation: twenty-seventh official adult lung and heart-lung transplant report—2010. J Heart Lung Transplant 2010; 29 (10) 1104-1118
    CrossrefPubMedGoogle Scholar
  • 105 Saggar R, Khanna D, Furst DE , et al. Systemic sclerosis and bilateral lung transplantation: a single centre experience. Eur Respir J 2010; 36 (4) 893-900
    CrossrefPubMedGoogle Scholar
  • 106 Schachna L, Medsger Jr TA, Dauber JH , et al. Lung transplantation in scleroderma compared with idiopathic pulmonary fibrosis and idiopathic pulmonary arterial hypertension. Arthritis Rheum 2006; 54 (12) 3954-3961
    CrossrefPubMedGoogle Scholar
  • 107 Sottile PD, Iturbe D, Katsumoto TR , et al. Outcomes in systemic sclerosis-related lung disease after lung transplantation. Transplantation 2013; 95 (7) 975-980
    CrossrefPubMedGoogle Scholar
  • 108 Khan IY, Singer LG, de Perrot M , et al. Survival after lung transplantation in systemic sclerosis. A systematic review. Respir Med 2013; 107 (12) 2081-2087
    CrossrefPubMedGoogle Scholar
  • 109 Takagishi T, Ostrowski R, Alex C, Rychlik K, Pelletiere K, Tehrani R. Survival and extrapulmonary course of connective tissue disease after lung transplantation. J Clin Rheumatol 2012; 18 (6) 283-289
    CrossrefPubMedGoogle Scholar
  • 110 De Cruz S, Ross D. Lung transplantation in patients with scleroderma. Curr Opin Rheumatol 2013; 25 (6) 714-718
    CrossrefPubMedGoogle Scholar
  • 111 Gasper WJ, Sweet MP, Golden JA , et al. Lung transplantation in patients with connective tissue disorders and esophageal dysmotility. Dis Esophagus 2008; 21 (7) 650-655
    CrossrefPubMedGoogle Scholar
  • 112 Tachikawa R, Tomii K, Ueda H , et al. Clinical features and outcome of acute exacerbation of interstitial pneumonia: collagen vascular diseases-related versus idiopathic. Respiration 2012; 83 (1) 20-27
    CrossrefPubMedGoogle Scholar
  • 113 Al-Hameed FM, Sharma S. Outcome of patients admitted to the intensive care unit for acute exacerbation of idiopathic pulmonary fibrosis. Can Respir J 2004; 11 (2) 117-122
    PubMedGoogle Scholar
  • 114 Mallick S. Outcome of patients with idiopathic pulmonary fibrosis (IPF) ventilated in intensive care unit. Respir Med 2008; 102 (10) 1355-1359
    CrossrefPubMedGoogle Scholar
  • 115 Mollica C, Paone G, Conti V , et al. Mechanical ventilation in patients with end-stage idiopathic pulmonary fibrosis. Respiration 2010; 79 (3) 209-215
    CrossrefPubMedGoogle Scholar
  • 116 Rangappa P, Moran JL. Outcomes of patients admitted to the intensive care unit with idiopathic pulmonary fibrosis. Crit Care Resusc 2009; 11 (2) 102-109
    PubMedGoogle Scholar
  • 117 Huynh TN, Weigt SS, Sugar CA, Shapiro S, Kleerup EC. Prognostic factors and outcomes of patients with pulmonary hypertension admitted to the intensive care unit. J Crit Care 2012; 27 (6) e7-e13
    CrossrefPubMedGoogle Scholar
  • 118 Churg A, Müller NL. Cellular vs fibrosing interstitial pneumonias and prognosis: a practical classification of the idiopathic interstitial pneumonias and pathologically/radiologically similar conditions. Chest 2006; 130 (5) 1566-1570
    CrossrefPubMedGoogle Scholar
  • 119 Singer JP, Blanc PD, Hoopes C , et al. The impact of pretransplant mechanical ventilation on short- and long-term survival after lung transplantation. Am J Transplant 2011; 11 (10) 2197-2204
    CrossrefPubMedGoogle Scholar
  • 120 Nosotti M, Rosso L, Tosi D , et al. Extracorporeal membrane oxygenation with spontaneous breathing as a bridge to lung transplantation. Interact Cardiovasc Thorac Surg 2013; 16 (1) 55-59
    CrossrefPubMedGoogle Scholar
  • 121 Baydur A. Mechanical ventilation in interstitial lung disease: which patients are likely to benefit?. Chest 2008; 133 (5) 1062-1063
    CrossrefPubMedGoogle Scholar
  • 122 Fernández-Pérez ER, Yilmaz M, Jenad H , et al. Ventilator settings and outcome of respiratory failure in chronic interstitial lung disease. Chest 2008; 133 (5) 1113-1119
    CrossrefPubMedGoogle Scholar
  • 123 Swigris JJ, Stewart AL, Gould MK, Wilson SR. Patients' perspectives on how idiopathic pulmonary fibrosis affects the quality of their lives. Health Qual Life Outcomes 2005; 3: 61
    CrossrefPubMedGoogle Scholar
  • 124 Mahler DA, Selecky PA, Harrod CG. Management of dyspnea in patients with advanced lung or heart disease: practical guidance from the American college of chest physicians consensus statement. Pol Arch Med Wewn 2010; 120 (5) 160-166
    PubMedGoogle Scholar
  • 125 Lewis D, Scullion J. Palliative and end-of-life care for patients with idiopathic pulmonary fibrosis: challenges and dilemmas. Int J Palliat Nurs 2012; 18 (7) 331-337
    CrossrefPubMedGoogle Scholar