Pulmonary Arterial Hypertension (PAH) as the Initial Manifestation of Systematic Lupus Erythematosus (SLE): A Rare Presentation

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5/23/20231 min read

Pulmonary arterial hypertension (PAH) can rarely present as the initial manifestation of systemic lupus erythematosus (SLE). SLE is a chronic autoimmune disease that can affect multiple organs and tissues in the body, including the lungs. PAH is a severe and potentially life-threatening condition characterized by high blood pressure in the arteries that supply the lungs.

Although PAH is not a common initial presentation of SLE, it can occur in some individuals. The exact cause of PAH in SLE is not fully understood, but it is thought to be related to the immune system's dysregulation and inflammation associated with SLE. The inflammation and immune dysfunction in SLE can lead to damage and remodeling of the blood vessels in the lungs, resulting in increased pulmonary arterial pressure and the development of PAH.

When PAH occurs as the initial manifestation of SLE, it can present with symptoms such as shortness of breath, fatigue, chest pain, dizziness, and fainting. However, these symptoms are not specific to PAH and can occur in various other conditions, making the diagnosis challenging. It requires a comprehensive evaluation by a healthcare professional, including a detailed medical history, physical examination, imaging studies, and laboratory tests.

The management of PAH as a result of SLE involves a multidisciplinary approach, usually involving a rheumatologist and a cardiologist or pulmonologist. Treatment may include medications to manage the symptoms and slow the progression of PAH, such as vasodilators, endothelin receptor antagonists, and prostacyclin analogs. Additionally, immunosuppressive therapies may be necessary to control the underlying autoimmune process of SLE.

It's important to note that PAH associated with SLE carries a higher risk of morbidity and mortality compared to idiopathic PAH. Therefore, early detection, accurate diagnosis, and prompt initiation of appropriate treatment are crucial in improving outcomes for individuals with this rare presentation.

If you suspect you or someone you know may have PAH or SLE, it is important to consult with a healthcare professional for a proper evaluation and diagnosis. They will be able to provide appropriate guidance and develop an individualized treatment plan based on the specific needs and circumstances.