Pleural Diseases in Connective Tissue Diseases

Abstract

Connective tissue diseases (CTD) are heterogeneous, immune-mediated inflammatory disorders often presenting with multiorgan involvement. With the advent of high-resolution computed tomography, CTD-related pleuritis—pleural thickening and effusion—is now increasingly recognized early in the disease trajectory. The natural history of CTD-related pleural effusions varies from spontaneous resolution to progressive fibrothorax with ventilatory impairment. Treatment of the underlying CTD is necessary to manage the pleural disease. Depending on the degree of symptom burden and physiological insult, specific treatment of pleural disease can include monitoring, repeated aspirations, systemic anti-inflammatory medication, and surgical decortication.

Keywords

connective tissue disease - collagen vascular disease - pleural disease - pleuritis - pleural effusion - pleural thickening - pneumothorax

Publikationsverlauf

Artikel online veröffentlicht:
28. März 2024

• References

• 1 Balbir-Gurman A, Guralnick L, Best LA, Nahir AM. Superior vena cava thrombosis and chylothorax in a young patient. Clin Exp Rheumatol 2005; 23 (4, Suppl 38): S106
• 2 Olson AL, Swigris JJ, Sprunger DB. et al. Rheumatoid arthritis-interstitial lung disease-associated mortality. Am J Respir Crit Care Med 2011; 183 (03) 372-378
• 3 Doyle TJ, Lee JS, Dellaripa PF. et al. A roadmap to promote clinical and translational research in rheumatoid arthritis-associated interstitial lung disease. Chest 2014; 145 (03) 454-463
• 4 Fingerman DL, Andrus FC. Visceral lesions associated with rheumatoid arthritis. Ann Rheum Dis 1943; 3 (03) 168-181
• 5 Horler AR, Thompson M. The pleural and pulmonary complications of rheumatoid arthritis. Ann Intern Med 1959; 51: 1179-1203
• 6 Walker WC, Wright V. Rheumatoid pleuritis. Ann Rheum Dis 1967; 26 (06) 467-474
• 7 Rockall AG, Rickards D, Shaw PJ. Imaging of the pulmonary manifestations of systemic disease. Postgrad Med J 2001; 77 (912) 621-638
• 8 Corcoran JP, Ahmad M, Mukherjee R, Redmond KC. Pleuro-pulmonary complications of rheumatoid arthritis. Respir Care 2014; 59 (04) e55-e59
• 9 Jurik AG, Graudal H. Pleurisy in rheumatoid arthritis. Scand J Rheumatol 1983; 12 (02) 75-80
• 10 Avnon LS, Abu-Shakra M, Flusser D, Heimer D, Sion-Vardy N. Pleural effusion associated with rheumatoid arthritis: what cell predominance to anticipate?. Rheumatol Int 2007; 27 (10) 919-925
• 11 Halla JT, Schrohenloher RE, Volanakis JE. Immune complexes and other laboratory features of pleural effusions: a comparison of rheumatoid arthritis, systemic lupus erythematosus, and other diseases. Ann Intern Med 1980; 92 (06) 748-752
• 12 Dodson WH, Hollingsworth JW. Pleural effusion in rheumatoid arthritis. Impaired transport of glucose. N Engl J Med 1966; 275 (24) 1337-1342
• 13 Ferreiro L, Alvarez-Dobaño JM, Valdés L. Systemic diseases and the pleura. Arch Bronconeumol 2011; 47 (07) 361-370
• 14 Potts DE, Willcox MA, Good Jr JT, Taryle DA, Sahn SA. The acidosis of low-glucose pleural effusions. Am Rev Respir Dis 1978; 117 (04) 665-671
• 15 Garcia-Zamalloa A, Ruiz-Irastorza G, Aguayo FJ, Gurrutxaga N. Pseudochylothorax. Report of 2 cases and review of the literature. Medicine (Baltimore) 1999; 78 (03) 200-207
• 16 Coe JE, Aikawa JK. Cholesterol pleural effusion. Report of 2 cases studied with isotopic techniques and review of the world literature. Arch Intern Med 1961; 108: 763-774
• 17 Hillerdal G. Chyliform (cholesterol) pleural effusion. Chest 1985; 88 (03) 426-428
• 18 Sassoon CS, Light RW. Chylothorax and pseudochylothorax. Clin Chest Med 1985; 6 (01) 163-171
• 19 Wrightson JM, Stanton AE, Maskell NA, Davies RJO, Lee YCG. Pseudochylothorax without pleural thickening: time to reconsider pathogenesis?. Chest 2009; 136 (04) 1144-1147
• 20 Amital A, Shitrit D, Adir Y. The lung in rheumatoid arthritis. Presse Med 2011; 40 (1 Pt 2): e31-e48
• 21 Faurschou P, Francis D, Faarup P. Thoracoscopic, histological, and clinical findings in nine case of rheumatoid pleural effusion. Thorax 1985; 40 (05) 371-375
• 22 Shaw M, Collins BF, Ho LA, Raghu G. Rheumatoid arthritis-associated lung disease. Eur Respir Rev 2015; 24 (135) 1-16
• 23 Sahn SA. State of the art. The pleura. Am Rev Respir Dis 1988; 138 (01) 184-234
• 24 Good Jr JT, King TE, Antony VB, Sahn SA. Lupus pleuritis. Clinical features and pleural fluid characteristics with special reference to pleural fluid antinuclear antibodies. Chest 1983; 84 (06) 714-718
• 25 Russell ML, Gladman DD, Mintz S. Rheumatoid pleural effusion: lack of response to intrapleural corticosteroid. J Rheumatol 1986; 13 (02) 412-415
• 26 Bouros D, Pneumatikos I, Tzouvelekis A. Pleural involvement in systemic autoimmune disorders. Respiration 2008; 75 (04) 361-371
• 27 Chapman PT, O'Donnell JL, Moller PW. Rheumatoid pleural effusion: response to intrapleural corticosteroid. J Rheumatol 1992; 19 (03) 478-480
• 28 Karkhanis VS, Joshi JM. Pleural effusion: diagnosis, treatment, and management. Open Access Emerg Med 2012; 4: 31-52
• 29 Shaikh MF, Jordan N, D'Cruz DP. Systemic lupus erythematosus. Clin Med (Lond) 2017; 17 (01) 78-83
• 30 Aguilera-Pickens G, Abud-Mendoza C. Pulmonary manifestations in systemic lupus erythematosus: pleural involvement, acute pneumonitis, chronic interstitial lung disease and diffuse alveolar hemorrhage. Reumatol Clín (Engl Ed) 2018; 14 (05) 294-300
• 31 Pego-Reigosa JM, Medeiros DA, Isenberg DA. Respiratory manifestations of systemic lupus erythematosus: old and new concepts. Best Pract Res Clin Rheumatol 2009; 23 (04) 469-480
• 32 Hannah JR, D'Cruz DP. Pulmonary complications of systemic lupus erythematosus. Semin Respir Crit Care Med 2019; 40 (02) 227-234
• 33 Torre O, Harari S. Pleural and pulmonary involvement in systemic lupus erythematosus. Presse Med 2011; 40 (1 Pt 2): e19-e29
• 34 Wang DY. Diagnosis and management of lupus pleuritis. Curr Opin Pulm Med 2002; 8 (04) 312-316
• 35 Blomgren SE, Condemi JJ, Vaughan JH. Procainamide-induced lupus erythematosus. Clinical and laboratory observations. Am J Med 1972; 52 (03) 338-348
• 36 Hepburn AL. The LE cell. Rheumatology (Oxford) 2001; 40 (07) 826-827
• 37 Keane MP, Lynch III JP. Pleuropulmonary manifestations of systemic lupus erythematosus. Thorax 2000; 55 (02) 159-166
• 38 Hooper C, Lee YC, Maskell N, Group BTSPG. BTS Pleural Guideline Group. Investigation of a unilateral pleural effusion in adults: British Thoracic Society Pleural Disease Guideline 2010. Thorax 2010; 65 (Suppl. 02) ii4-ii17
• 39 van den Hoogen F, Khanna D, Fransen J. et al. 2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League against Rheumatism collaborative initiative. Arthritis Rheum 2013; 65 (11) 2737-2747
• 40 Ferri C, Valentini G, Cozzi F. et al. Systemic Sclerosis Study Group of the Italian Society of Rheumatology (SIR-GSSSc). Systemic sclerosis: demographic, clinical, and serologic features and survival in 1,012 Italian patients. Medicine (Baltimore) 2002; 81 (02) 139-153
• 41 Thompson AE, Pope JE. A study of the frequency of pericardial and pleural effusions in scleroderma. Br J Rheumatol 1998; 37 (12) 1320-1323
• 42 Farrokh D, Abbasi B, Fallah-Rastegar Y, Mirfeizi Z. The extrapulmonary manifestations of systemic sclerosis on chest high resolution computed tomography. Tanaffos 2015; 14 (03) 193-200
• 43 Al-Jahdali H, Rajiah P, Allen C, Koteyar SS, Khan AN. Pictorial review of intrathoracic manifestations of progressive systemic sclerosis. Ann Thorac Med 2014; 9 (04) 193-202
• 44 Joseph J, Sahn SA. Connective tissue diseases and the pleura. Chest 1993; 104 (01) 262-270
• 45 Edwards Jr WG, Dines DE. Recurrent spontaneous pneumothorax in diffuse scleroderma. Report of a case. Dis Chest 1966; 49 (01) 96-98
• 46 De Zorzi E, Spagnolo P, Cocconcelli E. et al. Thoracic involvement in systemic autoimmune rheumatic diseases: pathogenesis and management. Clin Rev Allergy Immunol 2022; 63 (03) 472-489
• 47 Highland KB, Heffner JE. Pleural effusion in interstitial lung disease. Curr Opin Pulm Med 2004; 10 (05) 390-396
• 48 Taormina VJ, Miller WT, Gefter WB, Epstein DM. Progressive systemic sclerosis subgroups: variable pulmonary features. AJR Am J Roentgenol 1981; 137 (02) 277-285
• 49 Lee YH, Ji JD, Shim JJ, Kang KH, Song GG. Exudative pleural effusion and pleural leukocytoclastic vasculitis in limited scleroderma. J Rheumatol 1998; 25 (05) 1006-1008
• 50 Maeshima E, Nishimoto T, Yamashita M, Mune M, Yukawa S. Progressive systemic sclerosis-polymyositis overlap syndrome with eosinophilic pleural effusion. Rheumatol Int 2003; 23 (05) 252-254
• 51 Lin DF, Yan SM, Zhao Y. et al. Clinical and prognostic characteristics of 573 cases of primary Sjögren's syndrome. Chin Med J (Engl) 2010; 123 (22) 3252-3257
• 52 Strimlan CV, Rosenow III EC, Divertie MB, Harrison Jr EG. Pulmonary manifestations of Sjögren's syndrome. Chest 1976; 70 (03) 354-361
• 53 Alvarez-Sala R, Sánchez-Toril F, García-Martínez J, Zaera A, Masa JF. Primary Sjögren syndrome and pleural effusion. Chest 1989; 96 (06) 1440-1441
• 54 Kashiwabara K, Kishi K, Narushima K. et al. [Primary Sjögren's syndrome accompanied by pleural effusion]. Nihon Kyobu Shikkan Gakkai Zasshi 1995; 33 (11) 1325-1329
• 55 Ogihara T, Nakatani A, Ito H. et al. Sjögren's syndrome with pleural effusion. Intern Med 1995; 34 (08) 811-814
• 56 Suzuki H, Hickling P, Lyons CB. A case of primary Sjögren's syndrome, complicated by cryoglobulinaemic glomerulonephritis, pericardial and pleural effusions. Br J Rheumatol 1996; 35 (01) 72-75
• 57 Kawamata K, Haraoka H, Hirohata S, Hashimoto T, Jenkins RN, Lipsky PE. Pleurisy in primary Sjögren's syndrome: T cell receptor beta-chain variable region gene bias and local autoantibody production in the pleural effusion. Clin Exp Rheumatol 1997; 15 (02) 193-196
• 58 Tanaka A, Tohda Y, Fukuoka M, Nakajima S. [A case of Sjögren's syndrome with pleural effusion]. Nihon Kokyuki Gakkai Zasshi 2000; 38 (08) 628-631
• 59 Horita Y, Miyazaki M, Kadota J. et al. Type II diabetes mellitus and primary Sjögren's syndrome complicated by pleural effusion. Intern Med 2000; 39 (11) 979-984
• 60 Teshigawara K, Kakizaki S, Horiya M. et al. Primary Sjogren's syndrome complicated by bilateral pleural effusion. Respirology 2008; 13 (01) 155-158
• 61 Makimoto G, Asano M, Fujimoto N. et al. Bilateral pleural effusions as an initial presentation in primary Sjögren's syndrome. Case Rep Rheumatol 2012; 2012: 640353
• 62 Ma D, Lu H, Qu Y, Wang S, Ying Y, Xiao W. Primary Sjögren's syndrome accompanied by pleural effusion: a case report and literature review. Int J Clin Exp Pathol 2015; 8 (11) 15322-15327
• 63 Cruz-Pérez FdelP, Doval-Cortés A, Jaume-Anselmi F, Ramírez-Rivera J. Pleural effusion in a patient with primary Sjögren's syndrome successfully treated with corticosteroids. Bol Asoc Med P R 2015; 107 (03) 13-16
• 64 Ohe M, Hashino S, Ohara K. A case of primary Sjögren's syndrome with polyserositis. East J Med 2014; 19 (01) 54-57
• 65 Hosoda C, Hosaka Y, Ryu K, Kinoshita A, Saito K, Kuwano K. Pleuritis associated with primary Sjogren syndrome. Respirol Case Rep 2017; 6 (02) e00285
• 66 Ferreiro L, San José E, Suárez-Antelo J, Valdés L. Primary Sjögren syndrome with pleural effusion. Arch Bronconeumol 2017; 53 (10) 598-600
• 67 Shimoda M, Tanaka Y, Morimoto K. et al. Sjögren's syndrome with pleural effusion: difficult to distinguish from tuberculous pleurisy because of a high adenosine deaminase level. Intern Med 2022; 61 (04) 517-521
• 68 Fernandes Sr RL, Henriques AC, Ghiletchi A, Correia MI, Faria T. Undiagnosed primary Sjögren's syndrome with pleural involvement. Cureus 2023; 15 (09) e45981
• 69 Koga Y, Uchiyama Md K, Tsukamoto Md PhD N. et al. Primary Sjögren's syndrome with Waldenström's macroglobulinemia presenting as unilateral bloody pleural effusion. Respir Investig 2017; 55 (04) 283-286
• 70 Zintzaras E, Voulgarelis M, Moutsopoulos HM. The risk of lymphoma development in autoimmune diseases: a meta-analysis. Arch Intern Med 2005; 165 (20) 2337-2344
• 71 Rosenow E, Strimlan CV, Muhm JR, Ferguson RH. Pleuropulmonary manifestations of ankylosing spondylitis. Mayo Clin Proc 1977; 52 (10) 641-649
• 72 El Maghraoui A, Dehhaoui M. Prevalence and characteristics of lung involvement on high resolution computed tomography in patients with ankylosing spondylitis: a systematic review. Pulm Med 2012; 2012: 965956
• 73 Ersoy E, Akgol G, Ozgocmen S. Bilateral spontaneous pneumothorax in a patient with longstanding ankylosing spondylitis. Acta Reumatol Port 2014; 39 (04) 353-354
• 74 Wang CT, Tsen JC, Lin HJ, Cheng HH. Bilateral spontaneous pneumothorax in a patient with ankylosing spondylitis. Eur J Emerg Med 2007; 14 (02) 123-124
• 75 Kaneda H, Saito Y, Okamoto M, Maniwa T, Minami K, Imamura H. Bilaterally repeated spontaneous pneumothorax with ankylosing spondylitis. Gen Thorac Cardiovasc Surg 2007; 55 (06) 266-269
• 76 Lee CC, Lee SH, Chang IJ. et al. Spontaneous pneumothorax associated with ankylosing spondylitis. Rheumatology (Oxford) 2005; 44 (12) 1538-1541
• 77 Kanathur N, Lee-Chiong T. Pulmonary manifestations of ankylosing spondylitis. Clin Chest Med 2010; 31 (03) 547-554
• 78 Yao X, Abd Hamid M, Sundaralingam A. et al. Clinical perspective and practices on pleural effusions in chronic systemic inflammatory diseases. Breathe (Sheff) 2020; 16 (04) 200203
• 79 El Maghraoui A. Pleuropulmonary involvement in ankylosing spondylitis. Joint Bone Spine 2005; 72 (06) 496-502
• 80 Kinnear WJ, Shneerson JM. Acute pleural effusions in inactive ankylosing spondylitis. Thorax 1985; 40 (02) 150-151
• 81 Tanaka H, Itoh E, Shibusa T, Chiba H, Hirasawa M, Abe S. Pleural effusion in ankylosing spondylitis; successful treatment with intra-pleural steroid administration. Respir Med 1995; 89 (07) 509-511
• 82 Özden Sertçelik ÜKE, Babaoğlu E, Sarınç Ulaşlı S, Köksal D, Emri S. A rare cause of pleural effusion: ankylosing spondylitis. Acta Medica (Cordoba) 2018; 49 (02) 38-40
• 83 Erkan L, Uzun O, Findik S, Atici AG, Ozkaya S. Isolated pleural and pericardial effusion in a patient with ankylosing spondylitis. Respir Med 2007; 101 (02) 356-358
• 84 Ignacio García JM, Hita Pérez J, García Mendoza A, Alonso Atienza P, Miramón López J. Eosinofilia periférica y derrame pleural eosinofílico en un paciente con espondilitis anquilosante. Arch Bronconeumol 1987; 23 (03) 156-157
• 85 Narani A, Pirasath S. Acute bilateral pleural effusions in ankylosing spondylitis. Ceylon J Med Sci 2020; 56: 40
• 86 Yap KH, Choong CV, Ng Z, Albert LYH, Abisheganaden JA. Persistent pleural effusion and ankylosing spondylitis: a case report. Pleural Dis Case Rep 2018; 197: A3224
• 87 Edalatifard MRB, Kazemizadeh H, Boroofeh B, Dinparastisaleh R. Isolated pleural effusion in a patient with ankylosing spondylitis and his dramatic response to prednisolone. CRCP 2019; 3 (04) 123-127
• 88 Erkan F, Gül A, Tasali E. Pulmonary manifestations of Behçet's disease. Thorax 2001; 56 (07) 572-578
• 89 Davatchi F, Shahram F, Chams-Davatchi C. et al. Behcet's disease in Iran: analysis of 6500 cases. Int J Rheum Dis 2010; 13 (04) 367-373
• 90 Ceylan N, Bayraktaroglu S, Erturk SM, Savas R, Alper H. Pulmonary and vascular manifestations of Behcet disease: imaging findings. AJR Am J Roentgenol 2010; 194 (02) W158-64
• 91 Giannessi C, Smorchkova O, Cozzi D. et al. Behçet's disease: a radiological review of vascular and parenchymal pulmonary involvement. Diagnostics (Basel) 2022; 12 (11) 2868
• 92 Celenk C, Celenk P, Akan H, Başoğlu A. Pulmonary artery aneurysms due to Behçet's disease: MR imaging and digital subtraction angiography findings. AJR Am J Roentgenol 1999; 172 (03) 844-845
• 93 Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 51-1968. N Engl J Med 1968; 279 (25) 1385-1394
• 94 Austin A, Al-Faris F, Modi A, Chopra A. A transudative chylothorax associated with superior vena cava syndrome. Respir Med Case Rep 2019; 28: 100898
• 95 Cöplü L, Emrí S, Selçuk ZT. et al. Life threatening chylous pleural and pericardial effusion in a patient with Behçet's syndrome. Thorax 1992; 47 (01) 64-65
• 96 Husain SJ, Sadiq F, Zubairi AB, Khan JA. Massive unilateral chylous pleural effusion: a rare initial presentation of Behcet's disease. Singapore Med J 2006; 47 (11) 978-980
• 97 Oz N, Sarper A, Erdoğan A, Demircan A, Işin E. Video-assisted thoracic surgery for the management of pleural and pericardial effusion in Behçet's syndrome. Tex Heart Inst J 2000; 27 (03) 304-306
• 98 Kansu E, Ozer FL, Akalin E. et al. Behçet's syndrome with obstruction of the venae cavae. A report of seven cases. Q J Med 1972; 41 (162) 151-168
• 99 Kaşifoğlu T, Temiz G, Korkmaz C. Beneficial effect of talc pleurodesis in refractory pleural effusion due to Behçet's disease. Clin Exp Rheumatol 2007; 25 (06) 934
• 100 Alkhurassi HF, Ocheltree MR, Alsomali A, Alqunfoidi RA, Saadallah Sr A. Pleural effusion presenting in a young man with Behcet's disease. Cureus 2020; 12 (09) e10273
• 101 Kitching AR, Anders HJ, Basu N. et al. ANCA-associated vasculitis. Nat Rev Dis Primers 2020; 6 (01) 71
• 102 Moura CM, Branco C, Martins-Martinho J. et al. A glance into the future of anti-neutrophil cytoplasmic antibody-associated vasculitis. Ther Adv Musculoskelet Dis 2022; 14: 1-30
• 103 White J, Dubey S. Eosinophilic granulomatosis with polyangiitis: a review. Autoimmun Rev 2023; 22 (01) 103219
• 104 Szczeklik W, Sokołowska B, Mastalerz L. et al. Pulmonary findings in Churg-Strauss syndrome in chest X-rays and high resolution computed tomography at the time of initial diagnosis. Clin Rheumatol 2010; 29 (10) 1127-1134
• 105 Puéchal X. Granulomatosis with polyangiitis (Wegener's). Joint Bone Spine 2020; 87 (06) 572-578
• 106 Chin M, Leblanc A, Souza C. et al. A severe pleural complication associated with granulomatosis with polyangiitis. Respir Med Case Rep 2019; 28: 100933
• 107 Storelli E, Casali C, Natali P, Rossi G, Morandi U. Unusual pathogenesis of spontaneous pneumothorax secondary to Wegener's granulomatosis. Ann Thorac Surg 2007; 84 (01) 288-290
• 108 Shi X, Zhang Y, Lu Y. Risk factors and treatment of pneumothorax secondary to granulomatosis with polyangiitis: a clinical analysis of 25 cases. J Cardiothorac Surg 2018; 13 (01) 7
• 109 Oldroyd A, Lilleker J, Chinoy H. Idiopathic inflammatory myopathies - a guide to subtypes, diagnostic approach and treatment. Clin Med (Lond) 2017; 17 (04) 322-328
• 110 Le Goff B, Chérin P, Cantagrel A. et al. Pneumomediastinum in interstitial lung disease associated with dermatomyositis and polymyositis. Arthritis Rheum 2009; 61 (01) 108-118
• 111 Lega JC, Reynaud Q, Belot A, Fabien N, Durieu I, Cottin V. Idiopathic inflammatory myopathies and the lung. Eur Respir Rev 2015; 24 (136) 216-238
• 112 Pepmueller PH. Undifferentiated connective tissue disease, mixed connective tissue disease, and overlap syndromes in rheumatology. Mo Med 2016; 113 (02) 136-140
• 113 Ilan Y, Ben-Yehuda A, Okon E, Breuer R. Mixed connective tissue disease presenting as a left sided pleural effusion. Ann Rheum Dis 1992; 51 (10) 1157-1158
• 114 Cheah CK, Ramanujam S, Mohd Noor N, Gandhi C, D Souza BA, Gun SC. A case of mixed connective tissue disease with pseudo-pseudo Meigs' syndrome (PPMS)-like features. Lupus 2016; 25 (02) 214-216
• 115 Kundi M, Assad S, Babar S. et al. Mixed connective tissue disorder complicated by polymyositis, Sjogren's syndrome, pleural effusion and pericarditis. Cureus 2016; 8 (12) e906