Vasculopathy and pulmonary arterial hypertension

Area of Science:

• Rheumatology
• Immunology
• Vascular Medicine

Background:

• Vasculitis presents as primary or secondary to CTDs, infections, medications, or malignancy.
• Systemic Lupus Erythematosus (SLE) and Systemic Sclerosis (SS) are associated with significant vascular manifestations.
• Primary necrotizing vasculitides include Wegener's granulomatosis (WG), Churg-Strauss syndrome, and microscopic polyangiitis.

Purpose of the Study:

• To review the clinical presentation and management of vascular disease in SLE and SS.
• To discuss the epidemiology and treatment of pulmonary arterial hypertension (PAH) in SLE.
• To outline the characteristics and therapeutic strategies for primary necrotizing vasculitides.

Main Methods:

• Literature review of clinical presentations and management strategies for vasculitis.
• Analysis of prevalence and treatment outcomes for PAH in SLE patients.
• Summary of diagnostic markers and therapeutic responses for necrotizing vasculitides.

Main Results:

• Pulmonary arterial hypertension (PAH) affects 0.5-14% of SLE patients and responds well to immunosuppressants like cyclophosphamide and corticosteroids.
• Bosentan shows promise in improving PAH symptoms and quality of life in SLE.
• Necrotizing vasculitides, characterized by anti-neutrophil cytoplasmic antibodies, respond to induction therapy with cyclophosphamide and corticosteroids, with azathioprine (AZA) and methotrexate (MTX) for maintenance.

Conclusions:

• SLE-associated PAH requires prompt immunosuppressive treatment, with potential benefit from endothelin receptor antagonists.
• Primary necrotizing vasculitides necessitate aggressive initial therapy, followed by less toxic maintenance regimens.
• Future treatments for vasculitis may involve biologics like rituximab, infliximab, plasma exchange, and stem cell transplantation.