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Systemic Sclerosis (SSc)

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Complications

Systemic sclerosis (SSc) is a rare, autoimmune disorder in which widespread fibrosis and vasculopathy can affect various organ systems, including the skin, gastrointestinal tract, kidneys, lungs, and heart. Certain complications affecting these organ systems are more prevalent in individuals with SSc. Some of the major complications include gastric antral vascular ectasia (GAVE), intestinal pseudo-obstruction, scleroderma renal crisis (SRC), interstitial lung disease (ILD), pulmonary arterial hypertension (PAH), arrhythmias, pericardial diseases, myocardial dysfunction, and valvular diseases.1,2

Gastrointestinal Complications

Gastric Antral Vascular Ectasia

Gastric antral vascular ectasia (GAVE), also called “watermelon stomach,” is a potentially fatal complication that is diagnosed by gastroscopy. The distinguishing characteristics are dilated small vessels, erythematous stripes which radiate from the antrum and converge toward the pylorus, and longitudinal rugal folds in the antrum. These features together often resemble the stripes of a watermelon. 

GAVE may present with either chronic gradual occult gastrointestinal bleeding or acute heavy gastrointestinal bleeding resulting in iron deficiency or anemia. This complication can lead to malnutrition and weight loss.2,3

Intestinal Pseudo-Obstruction

Intestinal pseudo-obstruction is a serious, painful, and recurrent gastrointestinal complication that is reported in less than 10% of patients with SSc. As the name suggests, the movement of contents inside the intestinal lumen is blocked or delayed in the absence of a true obstruction. It presents with symptoms such as nausea, vomiting, bloating, abdominal distension and pain, small intestinal bacterial overgrowth, diarrhea, and constipation. It can sometimes be fatal.4

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Renal Complications

Renal complications in SSc may be acute or chronic, and they result in death in up to 10% of patients despite advances in therapy such as the introduction of angiotensin-converting enzyme (ACE) inhibitors.5

Scleroderma Renal Crisis

A major life-threatening renal complication of SSc is SRC, which occurs in 10% of patients with SSc, especially those with diffuse cutaneous SSc (dcSSc). SRC can occur following an abrupt onset of moderate-to-severe hypertension and lead to progressive renal failure. It often presents with manifestations of malignant hypertension, such as headache, blurred vision, confusion, and convulsions.5,6

Other clinical features include proteinuria, hematuria, acute congestive heart failure, and pulmonary edema. In around 50% of cases, microangiopathic hemolytic anemia with thrombocytopenia may occur. In 40% to 50% of patients, SRC can progress to end-stage renal failure that requires renal replacement therapy and, in some cases, kidney transplantation.5,6

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Pulmonary Complications

Interstitial Lung Disease

Interstitial lung disease is a pulmonary complication that is now the leading cause of mortality in patients with SSc. It often develops within the first 5 years following the first non-Raynaud phenomenon symptom, with 25% to 30% of patients developing progressive ILD. Clinical presentation at later stages includes overwhelming fatigue, nonproductive cough, dyspnea, nonspecific interstitial pneumonia (the most common pattern on computed tomography), and Velcro-like crackles on auscultation.7,8

Pulmonary Arterial Hypertension

Pulmonary arterial hypertension is a serious pulmonary complication, with the reported 3-year mortality ranging from 21% to 48%. It usually occurs several years after diagnosis, more commonly in patients with limited cutaneous SSc (lcSSc). Symptoms include dyspnea, chest pain, lower extremity swelling, lightheadedness, and syncope. Right heart catheterization is the standard method to confirm the diagnosis of PAH.1,2

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Cardiac Complications

Cardiac involvement is common in patients with SSc, accounting for mortality in approximately one-third of patients. SSc can affect all structures of the heart, including the pericardium, myocardium, conduction system, vasculature, and valves. Cardiac manifestations vary among patients, including primary features that are direct effects of the fibrotic process and secondary features that are due to PAH, ILD, or SRC. Severe life-threatening cardiac complications include conduction system abnormalities, coronary artery disease, arrhythmias, pericardial diseases, myocardial dysfunction (left and right ventricular dysfunction), valvular diseases, and heart failure.1,2,5

Because cardiac involvement is usually occult, every patient with SSc should routinely undergo standard 12-lead electrocardiography (ECG) and Doppler echocardiography to reduce the risk of sudden cardiac death. In patients with suspected arrhythmias, conduction defects, or other cardiac complications, evaluation tests such as 24-hour Holter monitoring, exercise testing, computed tomography, and cardiovascular magnetic resonance can also be performed.1,2,5

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Arrhythmias

The most frequent cardiac complications are arrhythmias, which include supraventricular arrhythmias, multiform ventricular premature beats, ventricular arrhythmias, left bundle branch block, and first-degree atrioventricular block. Common clinical manifestations of arrhythmias include fatigue, palpitations, syncope, and dizziness. If a patient is at high risk of sudden cardiac death, an implantable cardioverter defibrillator (ICD) may be required.9,10

Pericardial Diseases

The involvement of the pericardium leads to diseases such as pericarditis, pericardial effusions, cardiac tamponade, and constrictive pericarditis. However, pericardial disease is usually silent with the progression of SSc, and clinical symptoms such as chest pain, chest tightness, dyspnea, and fever present later in the course of disease.9,10

Myocardial Dysfunction

Myocardial dysfunction is among the most life-threatening cardiac dysfunctions and includes left ventricular dysfunction, right ventricular dysfunction, and congestive heart failure. Clinical symptoms include asymptomatic fatigue, lower leg edema, exertional dyspnea, and venous congestion.9,10

Valvular Diseases

The prevalence of valvular diseases is low in patients with SSc, with mitral valve prolapse being the most frequent valvular complication. Other valvular complications include tricuspid valve prolapse, aortic valve prolapse, degenerative aortic valve stenosis, and premature onset of severe aortic valve stenosis.9,10

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References

  1. Adigun R, Goyal A, Hariz A. Systemic sclerosis. In: StatPearls [Internet]. Treasure Island, FL: StatPearls Publishing; 2023. Updated May 8, 2022. Accessed May 1, 2023.
  2. Volkmann ER, Andréasson K, Smith V. Systemic sclerosis. Lancet. 2023;401(10373):304-318. doi:10.1016/S0140-6736(22)01692-0
  3. Morrisroe K, Hansen D, Stevens W, et al. Gastric antral vascular ectasia in systemic sclerosis: a study of its epidemiology, disease characteristics and impact on survival. Arthritis Res Ther. 2022;24(1):103. doi:10.1186/s13075-022-02790-1
  4. Luquez-Mindiola A, Atuesta AJ, Gómez-Aldana AJ. Gastrointestinal manifestations of systemic sclerosis: an updated review. World J Clin Cases. 2021;9(22):6201-6217. doi:10.12998/wjcc.v9.i22.6201
  5. Sobolewski P, Maślińska M, Wieczorek M, et al. Systemic sclerosis – multidisciplinary disease: clinical features and treatment. Reumatologia. 2019;57(4):221-233. doi:10.5114/reum.2019.87619.
  6. Reggiani F, Moroni G, Ponticelli C. Kidney involvement in systemic sclerosis. J Pers Med. 2022;12(7):1123. doi:10.3390/jpm12071123
  7. Perelas A, Silver RM, Arrossi AV, Highland KB. Systemic sclerosis-associated interstitial lung disease. Lancet Respir Med. 2020;8(3):304-320. doi:10.1016/S2213-2600(19)30480-1
  8. Khanna D, Tashkin DP, Denton CP, Renzoni EA, Desai SR, Varga J. Etiology, Risk Factors, and Biomarkers in Systemic Sclerosis with Interstitial Lung Disease. Am J Respir Crit Care Med. 2020;201(6):650-660. doi:10.1164/rccm.201903-0563CI
  9. Hung G, Mercurio V, Hsu S, Mathai SC, Shah AA, Mukherjee M. Progress in understanding, diagnosing, and managing cardiac complications of systemic sclerosis. Curr Rheumatol Rep. 2019;21(12):68. doi:10.1007/s11926-019-0867-0
  10. Nie LY, Wang XD, Zhang T, Xue J. Cardiac complications in systemic sclerosis: early diagnosis and treatment. Chin Med J (Engl). 2019;132(23):2865-2871. doi:10.1097/CM9.0000000000000535

Reviewed by Kyle Habet, MD, on 5/10/2023.

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