Severity and Prognosis for Scleroderma Pulmonary Involvement

LUNG (PULMONARY): MAIN MENU

This page was written by Shelley Ensz, and has not yet been medically edited. Scleroderma (SD) affects everyone differently. Just because something is listed here does not mean an individual patient will ever experience it. See Disclaimer.

Overview of Severity and Prognosis
Predictors of Morbidity and Mortality

Overview of Severity and Prognosis for Scleroderma Pulmonary Involvement

Lung involvement is the primary cause of scleroderma-related deaths today.

Changes in causes of death in systemic sclerosis, 1972-2002. Survival of scleroderma has changed since the treatment of renal crisis became possible. The change in pattern of scleroderma-related mortality over the past 30 years implicates the lung (both pulmonary hypertension and pulmonary fibrosis) as the primary causes of scleroderma related deaths today. PubMed. Ann Rheum Dis. 2007 Feb 28. (Also see: SSc: Prognosis and Mortality and Renal Involvement)

Outcome of patients with scleroderma admitted to intensive care unit. A report of nine cases. The outcome of scleroderma patients admitted to the ICU was extremely poor. Infectious complication was the most common cause of death in our patients. PubMed. Clin Exp Rheumatol. 2006 Jul-Aug;24(4):380-6. (Also see: SSc: Prognosis and Mortality)

Scleroderma Patients with Combined Pulmonary Hypertension and Interstitial Lung Disease. Patients with combined scleroderma lung disease features are more likely to have diffuse disease, represent older patients, and have a prognosis similar to individuals with isolated pulmonary hypertension, and may represent a distinct subpopulation of scleroderma. J Rheumatol. Volume 30: No. 11 November 2003;30:2398-405 (Also see: Pulmonary Hypertension, and Pulmonary Fibrosis)

Predictors of end stage lung disease in a cohort of patients with scleroderma. The risk can be predicted from baseline assessment of pulmonary function. In particular, those with normal pulmonary function at baseline are at very low risk. PubMed. Ann Rheum Dis 2003 Feb;62(2):146-50

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

It's a dry subject...but what is morbidity and mortality, anyway?

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.

Tissue Doppler Echocardiography and Cardiopulmonary Exercise Tests: The New Insight Into Heart and Respiratory Failure in Systemic Sclerosis Patients. Fibrosing of connective tissue in SSc patients leads to left ventricle diastolic dysfunction and restrictive ventilatory disturbances. This is a cause of severe cardiopulmonary system failure and exercise intolerance. W. Plazak. SAT0227 EULAR 2006. (Also see: Cardiac Involvement)

Homocysteine Plasma Concentration Is Related to Severity of Lung Impairment in Scleroderma. High level of homocysteinemia is associated with an increased risk of pulmonary disease in patients with scleroderma. Our data support the hypothesis that homocysteine could be involved in the pathogenetic process of scleroderma pulmonary involvement. The Journal of Rheumatology VOLUME 30: NO. 2 FEBRUARY 2003.

Severity of Capillaroscopic Abnormalities and Lung Disease Activity in Systemic Sclerosis. The severity of NCM (nailfold capillary microscopy) abnormalities is associated with end-organ damage in SSc. NCM may indicate the presence of active pulmonary disease (represented by areas of ground-glass opacities in high-resolution computed tomography) in SSc of relatively short duration. M. Bredemeier. FRI0084 EULAR 2003.

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.

Overview of Quality of Life (QOL)
Quality of Life with Scleroderma
Symptoms and QOL

Assessing QOL
Improving on the QOL
Personal Stories

The relationship of dyspnoea to function and quality of life (QoL) in systemic sclerosis (SSc). Interstitial lung disease contributes significantly to the sense of dyspnoea, function and health realted QoL in SSc. Pulmonary hypertension, assessed echocardiographically by the pulmonary artery systolic pressure, predicts the degree of dyspnoea but not function and HRQoL in SSc. M. Baron. Annals of the Rheumatic Diseases 2008;67:644-650. (Also see: Pulmonary Fibrosis, and Hypertension)

The Relative Contributions Of Pulmonary Artery Systolic Pressure (PASP) And Forced Vital Capacity (FVC) To Function And Health Related Quality Of Life (HRQOL) In Systemic Sclerosis (SSc). Although it is obvious from clinical practice that for patients with either severe PAH or ILD, lung disease may be an important cause of morbidity, in the average patient the degree of pulmonary involvement is not severe enough to interfere significantly with function or HRQoL. Disease severity outside the lung appears to be more important. M. Baron. AB0495 EULAR 2007. (Also see: Emotional Adjustment)