Vasculopathy and pulmonary arterial hypertension

Vasculitis can occur either as a primary condition or secondary to CTDs, infection, medication or malignancy. This article reviews the clinical presentation and management of vascular disease associated with SLE and SS, as well as the primary necrotizing vasculitides. Although pulmonary arterial hypertension (PAH) has traditionally been considered a rare complication of SLE, estimates of its prevalence range from 0.5% to 14% and it has a significant impact on prognosis. In contrast to PAH associated with other CTDs, patients with SLE respond well to immunosuppressive agents (cyclophosphamide in conjunction with corticosteroids). Improvements or stabilization of PAH symptoms and quality of life have also been observed with the oral, dual endothelin receptor antagonist, bosentan. SS is associated with a range of cutaneous and systemic signs of vasculitis. Immunosuppressive agents are effective, but are associated with an increased risk of lymphoma. The necrotizing vasculitides include WG, Churg–Strauss syndrome and microscopic polyangiitis, and are characterized by autoantibodies to neutrophil cytoplasmic constituents. WG is one of the most common forms of vasculitis; patients usually present with signs of respiratory disease. All three necrotizing vasculitides respond to cyclophosphamide and corticosteroids, while the less toxic AZA and MTX are effective for maintenance therapy. Future therapeutic approaches may include rituximab, plasma exchanges, the TNF antagonist infliximab and haematopoietic stem cell transplantation.