Severity and Prognosis for Scleroderma Pulmonary Involvement

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
Predictors of Morbidity and Mortality
Health Related Quality of Life Issues (HRQOL)


The primary cause of scleroderma-related death today is lung involvement; it used to be kidney failure, but that risk has been reduced thanks to dialysis and other treatments.

Prognosis of Pulmonary Hypertension in Systemic Sclerosis (Scleroderma). Early diagnosis and treatment substantially improves the prognosis for pulmonary hypertension associated with systemic sclerosis (scleroderma). ISN.

Overview of PH Prognosis
Functional Classification of PH
Markers for Prognosis of PH
Personal Stories of Prognosis of PH

Predictors of Morbidity and Mortality

Performance of candidate serum biomarkers for systemic sclerosis–interstitial lung disease (SSc–ILD). Our results show that lung–epithelial–derived surfactant protein is a relevant diagnostic biomarker of SSc-ILD, whereas glycoprotein Krebs von den Lungen-6 could assess lung fibrosis severity. PubMed, Arthritis Rheumatol. 01/09/2019.

Prediction of progression of interstitial lung disease (ILD) in patients with systemic sclerosis (SSc): the SPAR model. The evidence–based SPAR prediction model developed in our study might be helpful for the risk stratification of patients with mild SSc–ILD in clinical practice and cohort enrichment for future clinical trial design. PubMed, Ann Rheum Dis, 06/06/2018.

Interstitial Pneumonia with Autoimmune Features (IPAF): Overview of proposed criteria and recent cohort characterization. In this review, we summarize and compare previously proposed criteria to characterize these patients, provide an overview of the IPAF criteria and highlight recent investigations aimed at characterizing IPAF cohorts. PubMed, Clin Pulm Med, 2017 Sep;24(5):191-196.

Clinical Algorithms for the Diagnosis and Prognosis of Interstitial Lung Disease (ILD) in Systemic Sclerosis (SSc). Clinical algorithms had a good diagnostic performance for extents of SSc–ILD on high–resolution computed tomography (HRCT) with clinical and prognostic relevance (≥10 and ≥20%), and were also strongly related to mortality. Seminars in Arthritis and Rheumatism, 03/31/2017..

Prediction of pulmonary complications and long term survival in systemic sclerosis (SSc). This study provides data on long-term outcome of SSc and the timing and frequency of major organ complications. PubMed, Arthritis Rheumatol.

I'm confused! What exactly is the difference between morbidity and mortality?

Morbidity is another term for illness. A person can have several co-morbidities simultaneously. So, morbidities can range from Alzheimer's disease to cancer to traumatic brain injury. Morbidities are NOT deaths. Prevalence is a measure often used to determine the level of morbidity in a population. New York State Department of Health.

Mortality is another term for death. A mortality rate is the number of deaths due to a disease divided by the total population. If there are 25 lung cancer deaths in one year in a population of 30,000, then the mortality rate for that population is 83 per 100,000. New York State Department of Health.

So, morbidity just sounds deadly, but mortality really is.

Health Related Quality of Life Issues (HRQOL) for Scleroderma Pulmonary Involvement

Quality of Life with Scleroderma. The quality of life (QOL) is affected any time something prevents us from maintaining our normal routine or from doing something that we enjoy. A broken arm will affect your QOL on a temporary basis, but a chronic illness may it affect it indefinitely. ISN.

QOL with Scleroderma
Personal Stories
Go to Pulmonary Involvement Patient Stories

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