Interstitial Lung Disease in Systemic Sclerosis
Systemic sclerosis (SSc), sometimes referred to as systemic scleroderma, is a rare autoimmune condition. It results in the buildup of fibrosis in the skin and organs. This condition affects somewhere between 40,000 and 165,000 people in the United States alone. Females are four times as likely to be diagnosed with SSc than males. Some of the common symptoms are swelling, open sores, diarrhea, joint pain, Raynaud’s syndrome, heartburn, high blood pressure, and shortness of breath. It can also cause impaired function of the kidneys, heart, esophagus, and lungs when fibrosis spreads to those organs. Interstitial lung disease (ILD) is the term used to refer to fibrosis in the lungs.
ILD is unfortunately a frequent complication within SSc and it is often correlated to the severity of the condition. This means the diagnosis of ILD is typically associated with a worse prognosis for patients.
If we are able to detect ILD earlier, we will be able to provide patients better treatment and ultimately, improve their clinical outcomes.
A recent study conducted by the Ajou University College of Medicine in South Korea has Identified six clinical characteristics that could indicate ILD in SSc patients.
The Study
This study included 108 participants, all diagnosed with SSc. However, 45.4% (49 patients) had diffuse SSc while 54.6% (59 patients) had limited SSc. There were patients with ILD in both of these groups, but the incidence of the disease was higher in the diffuse group. All were between the ages of 14 and 78. The trial included 95 females and 13 males.
The goal was to evaluate the characteristics of all participants and determine any differences which may be present between those with ILD and those without. Researchers were successful in uncovering numerous characteristics which indicate clear correlation with ILD.
Unfortunately, this study also confirmed that disease duration was longer for patients who also had ILD. The average duration for those with SSc and ILD was 6.72 years versus just 4.28 years for those solely diagnosed with SSc.
Other characteristics associated with ILD included-
- Increased numbers of white blood cells (7,650.93 cells/microliter vs 6,468.96 cells/microliter)
- Higher platelet counts (282,100 platelets/microliter vs 241,300 platelets/microliter)
- Higher erythrocyte sedimentation rate (ESR) levels (39.4mm/hr vs 26.5 mm/hr)
- Pulmonary hypertension (PH) (25.6% vs 3.1%)
- More frequent positive anti-Scl70 antibody titer (62.8% vs 15.4%)
- Less frequent positive titer of anti-centromere antibody (9.3% vs 50.8%)
The most significant of these characteristics, as indicated by the researchers were PH, ESR, platelet counts, and the positive anti-Scl70 antibody.
This study was published in Archives of Rheumatology.
Hopefully the results from this study will lead to earlier diagnosis of ILD for SSc patients, and ultimately better outcomes.
You can read more about this study and what it could mean for systemic sclerosis patients here.
