Interstitial lung disease and pulmonary arterial hypertension in overlap syndrome: A case report
10.35460/2546-1621.2018-0042
- Author:
Mika Ana S FRIO
1
;
Sandra V NAVARRA
2
Author Information
1. Section of Rheumatology University of Santo Tomas Hospital Espana, Manila, Philippines
2. University of Santo Tomas Hospital Espana, Manila, Philippines
- Publication Type:Case Reports
- Keywords:
Overlap syndromel;
Scleroderma
- MeSH:
Pulmonary Arterial Hypertension;
Lung Diseases, Interstitial;
Lupus Erythematosus, Systemic
- From:
Journal of Medicine University of Santo Tomas
2019;3(1):309-312
- CountryPhilippines
- Language:English
-
Abstract:
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 profi le 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,
sildenafi l, 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, sildenafi l 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 infl ammatory vasculitis of SLE activity rather than fi brosis typical of SSc.
- Full text:1 JMUST 9.pdf
