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Systemic Sclerosis and Pulmonary Disease

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Lung Inflammation in Health and Disease, Volume I

Part of the book series: Advances in Experimental Medicine and Biology ((AEMB,volume 1303))

Abstract

Systemic sclerosis is a complex, often progressive, multisystem autoimmune disease. It is commonly categorized into limited cutaneous or diffuse cutaneous systemic sclerosis. There is near universal involvement of skin fibrosis and gastrointestinal dysfunction, but lung disease is not only common but also a most serious complication. Severe lung disease is the top cause of mortality, displacing scleroderma renal crisis as the leading cause of death. Whether there is limited cutaneous or diffuse cutaneous manifestations can be predictive of what type of lung disease that can present in the patient. Limited cutaneous systemic sclerosis patients tend to have pulmonary hypertension whereas diffuse cutaneous systemic sclerosis patients tend to have interstitial lung disease. There are more rare phenotypes associated with antibodies Th/To and U3RNP that can have both pulmonary hypertension and interstitial lung disease concomitantly. There are inherent challenges in the management for both pulmonary hypertension and interstitial lung disease but with the focus on early diagnosis for each of these lung complications, treatment may have a higher chance of efficacy.

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Abbreviations

ACA:

anticentromere antibody

ACEI:

angiotensin converting enzyme inhibitor

ACR:

American College of Rheumatology

ANA:

antinuclear antibody

dcSSc:

diffuse cutaneous systemic sclerosis

ECG:

electrocardiogram

EULAR:

European League Against Rheumatism

EUSTAR:

EULAR Scleroderma Trials and Research group

GERD:

gastrointestinal reflux

GIT:

gastrointestinal tract

ILD:

interstitial lung disease

lcSSc:

limited cutaneous systemic sclerosis

MAHA:

microangiopathic hemolytic anemia

MRI:

magnetic resonance imaging

mRSS:

modified Rodnan skin score

PAH:

pulmonary arterial hypertension

PH:

pulmonary hypertension

RNP:

ribonucleoprotein

SIBO:

small intestinal bacterial overgrowth

SMR:

standardized mortality ratios

SRC:

scleroderma renal crisis

SSC:

scleroderma

ssSSc:

systemic sclerosis sine scleroderma

Scl70:

Scleroderma 70

RNAP III:

ribonuclear antigen polymerase III

Pm-Scl:

polymyositis scleroderma

AA:

African American

PFT:

pulmonary function test

DLco:

diffusion capacity of the lungs for carbon monoxide

MCTD:

mixed connective tissue disease

SLE:

systemic lupus erythematosus

FVC:

forced vital capacity

WHO:

World Health Organization

NT-proBNP:

N-terminal probrain natriuretic peptide

TTE:

transthoracic echo

RHC:

right heart catheterization

mPAP:

mean pulmonary artery pressure

TR:

tricuspid regurgitation

PF:

pulmonary fibrosis

RLD:

restrictive lung disease

6MWT:

6-min walk test

SLS:

Scleroderma Lung Study

MMF:

mycophenolate mofetil

AHCT:

autologous hematopoietic stem cell transplant

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Authors and Affiliations

  1. Division of Rheumatology, Department of Medicine, Albany Medical College, Albany, NY, USA

    Khoa Ngo

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Correspondence to Khoa Ngo .

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  1. Department of Molecular & Cellular Physiology, Albany Medical College, Albany, NY, USA

    Yong-Xiao Wang

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Ngo, K. (2021). Systemic Sclerosis and Pulmonary Disease. In: Wang, YX. (eds) Lung Inflammation in Health and Disease, Volume I. Advances in Experimental Medicine and Biology, vol 1303. Springer, Cham. https://doi.org/10.1007/978-3-030-63046-1_10

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