Can scleroderma cause bursting veins?

Scleroderma and Vascular Complications: Risk of Bursting Veins

Yes, scleroderma can cause vascular damage that may lead to blood vessel abnormalities, including the potential for vessel rupture, though frank "bursting veins" is not a typical primary manifestation of the disease. 1, 2

Pathophysiology of Vascular Involvement in Scleroderma

• Scleroderma is fundamentally characterized by three distinct pathologic processes: fibrosis, autoimmunity, and specific vascular changes that are best described as a vasculopathy rather than vasculitis 3
• The vascular disease in scleroderma plays a fundamental role in its pathogenesis and associated tissue injury 1
• Endothelial cell injury can be induced by various mechanisms including infection, immune-mediated cytotoxicity, antiendothelial antibodies, and ischemia-reperfusion 1
• Blood vessel abnormalities in scleroderma include characteristic non-inflammatory macrovascular and microvascular changes with formation of thickened neointima that can be occlusive 2

Specific Vascular Manifestations in Scleroderma

• Nearly all patients with systemic sclerosis have Raynaud phenomenon, and approximately half develop digital ulcers due to vascular compromise 4
• Vascular damage in scleroderma leads to reduced blood vessel density in affected tissues, with studies showing diminished capillary density in scleroderma skin 5
• The reduction in blood vessels occurs early in the disease evolution, preceding the appearance of intense fibrosis 5
• Pulmonary arterial hypertension (PAH) is a serious vascular complication occurring in approximately 16% of scleroderma patients and is responsible for up to 50% of deaths in patients with limited scleroderma 6

Potential for Vessel Rupture

• While not commonly described as "bursting veins," the vascular fragility in scleroderma can lead to complications such as telangiectasias (dilated blood vessels visible on the skin) 7
• In cardiac involvement of scleroderma, linear mid-myocardial enhancement can be seen in 66% of patients, either in the ventricular septum or left ventricular free wall, indicating vascular damage 4
• The combination of vessel wall abnormalities and fibrosis can lead to compromised vascular integrity 3
• Vascular malformation and rarefaction may result from systemic angiogenic dysregulation, with overexpression of vascular endothelial growth factor (VEGF) but improper interactions with smooth muscle cells needed to stabilize blood vessels 3

Management of Vascular Complications

• Treatment of Raynaud phenomenon in systemic sclerosis typically begins with dihydropyridine calcium channel blockers (especially nifedipine), followed by phosphodiesterase 5 inhibitors or intravenous iloprost if needed 4
• Bosentan can reduce the development of new digital ulcers in scleroderma patients 4
• Regular screening for pulmonary hypertension is recommended for all scleroderma patients, particularly those with limited cutaneous disease 6
• Early intervention with vasoactive therapy is recommended upon diagnosis of systemic scleroderma to address vascular complications 7

Clinical Monitoring and Prognosis

• Doppler echocardiography is the primary non-invasive screening tool for pulmonary hypertension in scleroderma 6
• A marked decrease in diffusing capacity (DLCO ≤55% of predicted) is associated with a 35% risk of eventually developing pulmonary arterial hypertension 6
• Older men with scleroderma, those with rapid rises in systolic pulmonary artery pressure, or those with initial sPAP >60 mm Hg are at greatest risk of dying from PAH 6
• The vascular damage in scleroderma tends to be progressive, with early intervention potentially slowing but not necessarily reversing the process 2

While scleroderma does cause significant vascular pathology that can lead to vessel damage, the classic presentation of "bursting veins" is not typically described in the medical literature as a primary manifestation of the disease. The vascular complications more commonly manifest as Raynaud's phenomenon, digital ulcers, telangiectasias, and pulmonary arterial hypertension.