Scleroderma
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Scleroderma: Organ Impact Overview
Scleroderma significantly affects various organs. Lung complications are prevalent, heart issues range in severity, and GI complications impact nearly all patients, lowering life quality and increasing mortality risk.
Lung, heart, and GI systems are severely impacted, with high prevalence and serious consequences in scleroderma.
The Impact of Pulmonary Issues in Systemic Sclerosis
Pulmonary complications are a significant concern in systemic sclerosis (SSc), affecting over 80% of patients. These include interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH).
ILD often starts with shortness of breath and coughing, gradually becoming more severe.
PAH can initially be asymptomatic but eventually leads to major breathing difficulties. Pulmonary issues are common after esophageal involvement in SSc patients.
ILD often starts with shortness of breath and coughing, gradually becoming more severe.
PAH can initially be asymptomatic but eventually leads to major breathing difficulties. Pulmonary issues are common after esophageal involvement in SSc patients.
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Source Quotes:
Pulmonary involvement occurs in over 80 percent of patients with systemic sclerosis (SSc) and is second in frequency only to esophageal involvement as a visceral complication.,SSc-associated ILD typically presents with the subacute onset of dyspnea on exertion and sometimes nonproductive cough.,Pulmonary hypertension (PH) is the most common pulmonary vascular disease in SSc.
Heart Complications in Systemic Sclerosis
Cardiac issues in systemic sclerosis (SSc) can range from unnoticed to life-threatening, affecting various parts of the heart. These can include the heart muscle and its electrical system.
Symptoms like irregular heartbeats and heart muscle damage can lower the heart's performance.
Fluid around the heart, or pericardial involvement, shows how widely the heart can be affected in SSc.
Symptoms like irregular heartbeats and heart muscle damage can lower the heart's performance.
Fluid around the heart, or pericardial involvement, shows how widely the heart can be affected in SSc.
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Source Quotes:
Cardiac involvement is frequent in SSc but can be entirely asymptomatic.,All anatomic domains of the heart can be affected in patients with SSc, including the myocardium, pericardium, and conduction system.
Heart problems may include: Abnormal heart rhythm, Fluid around the heart, Fibrosis in heart muscle, decreasing heart function.
Digestive System Challenges in Scleroderma
Nearly 90% of SSc patients experience gastrointestinal (GI) problems, greatly impacting their quality of life. These issues can range from heartburn and trouble swallowing to serious conditions like malabsorption due to bacterial growth.
Problems in the digestive system can worsen lung issues, especially through aspirations caused by esophageal dysfunction.
Problems in the digestive system can worsen lung issues, especially through aspirations caused by esophageal dysfunction.
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Source Quotes:
Nearly 90 percent of patients with systemic sclerosis (SSc) have some degree of gastrointestinal (GI) involvement, and approximately one-half are symptomatic.,The presence of malabsorption and esophageal dysfunction among patients with SSc is associated with an unfavorable prognosis because they can exacerbate other manifestations of the disease, notably lung fibrosis due to microaspiration and cardiac manifestations due to poor nutrition and electrolyte abnormalities.
Recurrent aspiration due to esophageal dysmotility and chronic gastroesophageal reflux can lead to aspiration of pharyngeal and gastric contents, which may contribute to lung disease in SSc.
Kidney Complications and Scleroderma Renal Crisis
Renal (kidney) complications, especially scleroderma renal crisis (SRC), although less common, are serious in scleroderma patients. SRC can be life-threatening, leading to sudden kidney issues and high blood pressure.
This condition might require dialysis in 20-50% of patients. Kidney problems can also exist without obvious symptoms but are often found during autopsies of those affected.
This condition might require dialysis in 20-50% of patients. Kidney problems can also exist without obvious symptoms but are often found during autopsies of those affected.
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Source Quotes:
The most serious kidney manifestation of SSc is scleroderma renal crisis (SRC), which occurs in a minority of patients.,SRC is characterized by acute kidney injury (AKI), abrupt onset of moderate to marked hypertension, and a normal urine sediment without heavy proteinuria.,Approximately 20 to 50 percent of patients with SRC will require dialysis.
Life-threatening renal involvement called SRC develops in up to 10 percent of patients and is far more frequent in patients with dcSSc than lcSSc.
Key Takeaways
Conclusions
Scleroderma significantly impacts multiple organ systems, with pulmonary complications affecting the majority of patients. Issues such as interstitial lung disease and pulmonary arterial hypertension emerge as serious concerns due to their progressive nature.
Cardiac challenges, ranging from mild arrhythmias to severe muscle and electrical dysfunction, further complicate the disease's course. Meanwhile, gastrointestinal problems, experienced by nearly all patients, severely diminish life quality.
Renal involvement, though less common, poses serious risks with potential for kidney failure. This underscores the need for vigilant monitoring and timely intervention.
Cardiac challenges, ranging from mild arrhythmias to severe muscle and electrical dysfunction, further complicate the disease's course. Meanwhile, gastrointestinal problems, experienced by nearly all patients, severely diminish life quality.
Renal involvement, though less common, poses serious risks with potential for kidney failure. This underscores the need for vigilant monitoring and timely intervention.
Literature Review
D'Andrea, 2004
Organ Impact:
Scleroderma affects heart and lungs: The study highlights that scleroderma impacts both heart and lung functions significantly.
Diastolic dysfunction linked to severity: Right ventricular diastolic dysfunction is associated with more severe forms of scleroderma.
Assessment tools predict disease severity: Doppler myocardial imaging helps identify patients at higher risk of severe scleroderma.
Literature Review
Kim H, 2021
Study Focus:The study reviews mortality and morbidity associated with scleroderma renal crisis and examines trends over time.
Patient Outcomes:
Mortality Trends: Mortality improved by 50% after angiotensin-converting enzyme inhibitors were introduced, but no further advancements since.
Renal Transplants vs Dialysis: Survival rates are higher for patients post-transplant (54%-91%) than for those on dialysis (31%-56%).
Unmet Needs:High morbidity and mortality persist without significant post-angiotensin-converting enzyme inhibitor advancements, indicating a need for novel treatments.
Literature Review
Vanhaecke et al., 2020
Main Findings:
Coexistence rate of SSc and LoS: Ranges between 2.4% and 7.4% in studies analyzed.
Common traits in coexistence: Raynaud's phenomena often present; specific antibodies observed.
Study Cohort Insights: 2.7% coexistence in 296 patients at Ghent University.
Significance:First systematic study showing high overlap of SSc and LoS, despite rarity.