Interstitial Lung Disease and Pulmonary Arterial Hypertension in Overlap Syndrome: A Case Report
Mika Ana S. Frio, Sandra V. Navarra
Apr 2019 DOI 10.35460/2546-1621.2018-0042 Access
OBJECTIVE: To present the onset of severe pulmonary arterial hypertension (PAH) in a patient with interstitial lung disease (ILD) associated with overlap syndrome.
CASE PRESENTATION: A 42-year-old female was diagnosed with overlap syndrome consisting of systemic lupus erythematosus (SLE), systemic sclerosis (SSc) and rheumatoid arthritis (RA). The serologic profile included positive antinuclear antibody (ANA), anti-dsDNA, anti-RNP, anti-Ro, anti-Scl70, anti-Sm, rheumatoid factor and hypocomplementemia (C3, C4). She had chronic stable ILD for 17 years maintained on hydroxychloroquine (HCQ), prednisone 5 mg/day and indacaterol. The current admission was due to progressive dyspnea and right-sided heart failure over the past month. Chest radiograph showed pulmonary congestion, and 2-dimensional echocardiography (2DE) disclosed severe PAH with systolic pulmonary arterial pressure (SPAP) of 76 mmHg by tricuspid regurgitation (TR) jet, dilated right ventricle (RV) with poor systolic function, moderate pericardial effusion with no signs of tamponade. She received furosemide, beraprost, sildenafil, and prednisone was increased to 20 mg/day. Two weeks following discharge, there was complete resolution of symptoms and repeat 2DE showed non-dilated RV with good systolic function, normal SPAP of 21.4 mmHg and minimal pericardial effusion. Prednisone was tapered to 5 mg/day; beraprost, sildenafil and HCQ were continued.
CONCLUSION: Overlap syndrome was diagnosed by the combination of clinical features and serology distinctive of SLE, SSc and RA. Her illness, particularly ILD, was adequately controlled over several years, until the recent onset of PAH complicated by right-sided heart failure. The dramatic response to high-dose steroids is more consistent with inflammatory vasculitis of SLE activity rather than fibrosis typical of SSc.
- Firestein GS, Gabriel SE, McInnes IB, O’Dell JR. Kelley and Firestein’s Textbook of Rheumatology. 2017.
- Gaubitz M. Epidemiology of connective tissue disorders. Rheumatology. 2006;45(3):iii3–iii4.
- Kahler C. Pulmonary arterial hypertension (PAH) in connective tissue diseases. Rheumatology. 2006 Oct 1;45(3):iii11–iii13.
- Condliffe R, Howard LS. Connective tissue disease-associated pulmonary arterial hypertension. F1000Prime Rep [Internet]. [cited 2015 Jan 5;7]. Available from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4311276/
- Mathai SC, Hassoun PM. Pulmonary arterial hypertension in connective tissue diseases. Heart Fail Clin. 2012 Jul;8(3):413–25.
- Mittoo S, Jacob T, Craig A, Bshouty Z. Treatment of pulmonary hypertension in patients with connective tissue disease and interstitial lung disease. Can Respir J, J Can Thorac Soc. 2010;17(6):282–6.
- Jury E, D’Cruz D, Morrow W. Autoantibodies and overlap syndromes in autoimmune rheumatic disease. J Clin Pathol. 2001 May;54(5):340–47.
- Lockshin MD, Levine AB, Erkan D. Patients with overlap autoimmune disease differ from those with “pure” disease. Lupus Sci Med. 2015 May 1;2(1):e000084.
- Sharma S, Kumar U. Scleroderma overlap syndromes. Int J Rheum Dis. 2016 Sep 1;19(9):831–3.
- Horimoto AMC, da Costa IP. Overlap between systemic sclerosis and rheumatoid arthritis: a distinct clinical entity? Rev Bras Reumatol Engl Ed. 2016 Jul 1;56(4):287–98.
- Thorne I, Piercy C. Early scleroderma overlap syndrome (pulmonary hypertension, ILD, polymyositis and inflammatory arthritis) and an unplanned pregnancy. Rheumatology Advances in Practice. 2017 Oct;1(1).
- Turkcapar N, Olmez U, Ozer D, Duzgun N, Duman M. A case of overlap syndrome with rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis and secondary Sjögren’s syndrome. Rheumatol Int. 2006 Jul;26(9):841–5.
- Shaw M, Collins BF, Ho LA, Raghu G. Rheumatoid arthritis-associated lung disease. Eur Respir Rev. 2015 Mar 1;24(135):1–16.
- Kim EJ, Collard HR, King TE. Rheumatoid arthritis-associated interstitial lung disease. Chest. 2009 Nov;136(5):1397–405.
- Szücs G, Szekanecz Z, Zilahi E, Kapitány A, Baráth S, Szamosi S, et al. Systemic sclerosis–rheumatoid arthritis overlap syndrome: a unique combination of features suggests a distinct genetic, serological and clinical entity. Rheumatology. 2007 Jun 1;46(6):989–93.
- Herzog EL, Mathur A, Tager AM, Feghali-Bostwick C, Schneider F, Varga J. Interstitial lung disease associated with systemic sclerosis and idiopathic pulmonary fibrosis. Arthritis Rheumatol. Hoboken, NJ. 2014 Aug;66(8):1967–78.
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