DOI: http://dx.doi.org/10.18203/2320-6012.ijrms20195645

Usual interstitial pneumonia - secondary vs idiopathic pulmonary fibrosis

Divyendu Sharma, Rajesh Agrawal, Rajat Agarwal, Amit Kumar, Utkarsh Gupta

Abstract


Background: Interstitial Lung Diseases is a group of disorders where the pulmonary interstitium, alveolar structures and the small airways are affected. Identification of a specific pattern on HRCT, with a thorough clinical evaluation can help a physician in narrowing down the differential diagnosis for the underlying cause. Usual Interstitial Pneumonia (UIP) is a frequently identified pattern. Differentiating patients with definite UIP pattern, into IPF and non-IPF spectrums is important. Aim of this study is to compare UIP patients with a secondary cause vs Idiopathic Pulmonary Fibrosis.

Methods: Statistically 33 patients having UIP pattern on HRCT were evaluated based on the history of extrapulmonary symptoms, environmental exposure, drugs and subsequent serology testing. Patients were divided into two groups - IPF and UIP with a secondary cause. Both groups were compared on various clinical parameters. Inferences were drawn from the same.

Results: Total 66.6% patients were identified to have Idiopathic Pulmonary Fibrosis, 33.3% had UIP with a secondary cause. Majority of patients with a secondary cause had Connective Tissue Disorder (90.9%) and one patient of Chronic Hypersensitivity Pneumonitis (HP).

Conclusions: Absence of extrapulmonary symptoms in UIP patients need no further investigations and can be diagnosed as a case of IPF. However, presence of extrapulmonary symptoms needs further evaluation to diagnose the underlying disease and start treatment for the same.


Keywords


Connective tissue disorder, Idiopathic pulmonary fibrosis, Usual interstitial pneumonia, Usual interstitial pneumonia with a secondary cause

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References


Jay h. Ryu, Moisés Selman, Thomas V. Colby, Talmadge E. King, Jr. Idiopathic Interstitial Pneumonias; In: Murray and Nadel’s Textbook of Respiratory Medicine, 5th ed. Elsevier; 2015:1118-1120.

Raghu G, Remy-Jardin M, Myers JL, Richeldi L, Ryerson CJ, Lederer DJ, et al. Diagnosis of idiopathic pulmonary fibrosis. An official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respira Crit Care Med. 2018 Sep 1;198(5):e44-68.

Park JH, Kim DS, Park IN, Jang SJ, Kitaichi M, Nicholson AG, et al. Prognosis of fibrotic interstitial pneumonia: idiopathic versus collagen vascular disease–related subtypes. Am J Respira Crit Care Med. 2007 Apr 1;175(7):705-11.

Singh S, Collins BF, Sharma BB, Joshi JM, Talwar D, Katiyar S, et al. Interstitial lung disease in India. Results of a prospective registry. Am J Respira Crit Care Med. 2017 Mar 15;195(6):801-13.

Martinez FJ, Flaherty K. Pulmonary function testing in idiopathic interstitial pneumonias. Proceed Am Thoracic Soc. 2006 Jun;3(4):315-21.

Aletaha D, Neogi T, Silman AJ, Funovits J, Felson DT, Bingham III CO, et al. 2010 rheumatoid arthritis classification criteria: an American College of Rheumatology/European League Against Rheumatism collaborative initiative. Arthritis Rheumatism. 2010 Sep;62(9):2569-81.

van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, et al. Classification criteria for systemic sclerosis: an ACR-EULAR collaborative initiative. Arthritis Rheumatism. 2013 Nov;65(11):2737.

Shiboski CH, Shiboski SC, Seror R, Criswell LA, Labetoulle M, Lietman TM, et al. 2016 American College of Rheumatology/European League Against Rheumatism classification criteria for primary Sjögren's syndrome: a consensus and data‐driven methodology involving three international patient cohorts. Arthritis Rheumatol. 2017 Jan;69(1):35-45.

Vasakova M, Morell F, Walsh S, Leslie K, Raghu G. Hypersensitivity pneumonitis: perspectives in diagnosis and management. Am J Respira Crit Care medicine. 2017 Sep 15;196(6):680-9.

American Thoracic Society. Idiopathic pulmonary fibrosis: diagnosis and treatment: international consensus statement. Am J Respir Crit Care Med. 2000;161:646-64.

Kärkkäinen M, Kettunen HP, Nurmi H, Selander T, Purokivi M, Kaarteenaho R. Effect of smoking and comorbidities on survival in idiopathic pulmonary fibrosis. Respiratory Res. 2017 Dec;18(1):160.

Baumgartner KB, Samet JM, Stidley CA, Colby TV, Waldron JA. Cigarette smoking: a risk factor for idiopathic pulmonary fibrosis. Am J Respire Crit Care Med. 1997 Jan;155(1):242-8.

Lahita RG. The connective tissue diseases and the overall influence of gender. Inter J Fertility Menopausal Studies. 1996;41(2):156-65.

Lee JS. Gastroesophageal Reflux and IPF. In Idiopathic Pulmonary Fibrosis. Humana Press: Cham; 2019:379-387

Tobin RW, POPE CE, Pellegrini CA, Emond MJ, Sillery JI, Raghu G. Increased prevalence of gastroesophageal reflux in patients with idiopathic pulmonary fibrosis. Am J Respira Crit Care Med. 1998 Dec 1;158(6):1804-8.

Christmann RB, Wells AU, Capelozzi VL, Silver RM. Gastroesophageal reflux incites interstitial lung disease in systemic sclerosis: clinical, radiologic, histopathologic, and treatment evidence. In: Seminars in arthritis and rheumatism. WB Saunders. 2010;40(3):241-9.

Spicknall KE, Zirwas MJ, English III JC. Clubbing: an update on diagnosis, differential diagnosis, pathophysiology, and clinical relevance. J Am Acad Dermatol. 2005 Jun 1;52(6):1020-8.

van Manen MJ, Vermeer LC, Moor CC, Vrijenhoeff R, Grutters JC, Veltkamp M, et al. Clubbing in patients with fibrotic interstitial lung diseases. Respira Med. 2017 Nov 1;132:226-31.

Flaherty KR, Colby TV, Travis WD, Toews GB, Mumford J, Murray S, et al. Fibroblastic foci in usual interstitial pneumonia: idiopathic versus collagen vascular disease. Am J Respira Crit Care Med. 2003 May 15;167(10):1410-5.

Shelton DN, Chang E, Whittier PS, Choi D, Funk WD. Microarray analysis of replicative senescence. Curr Biol. 1999 Sep 9;9(17):939-45.