When the immune system strikes twice: SLE-associated pulmonary arterial hypertension: a case report
Abstract
Background: Pulmonary arterial hypertension (PAH) is a rare but potentially severe manifestation in patients with connective tissue diseases (CTDs) like systemic lupus erythematosus (SLE). While immunosuppressive therapy is not indicated in most PAH subtypes, it plays a pivotal role in SLE-associated PAH due to its immune-mediated pathophysiology.
Case summary: This case report describes a 43-year-old woman with SLE who experienced two severe PAH flares, each following discontinuation of maintenance immunosuppression. The first episode was complicated by circulatory shock following β-blocker administration and treated with a combination of corticosteroids and pulmonary vasodilators. The second episode responded well to immunosuppressive therapy alone, without the need to escalate pulmonary vasodilator treatment.
Discussion: Both SLE-PAH flares followed discontinuation of maintenance immunosuppressive therapy, underscoring that continuous immunosuppression might reduce flare risk. The clinical course during flares aligns with evidence that SLE-associated PAH benefits from immunosuppression, in contrast to other PAH forms. Finally, the case highlights that negative chronotropic-inotropic agents (e.g. β-blockers) can induce life-threatening haemodynamic shock in patients with PAH.
How strong is the evidence?
Evidence Hub reads this as a case report, done in people. Its evidence level is 5 of 5 (Very low · case / narrative / animal / in vitro / in silico). Case reports, narrative reviews, and animal, cell or computer-model studies give the weakest evidence for human health.
Read the paper
Related studies
- Drug repositioning: a brief overview
- Cyclic nucleotide phosphodiesterases in Drosophila melanogaster
- Time-resolved in silico modeling of fine-tuned cAMP signaling in platelets: feedback loops, titrated phosphorylations and pharmacological modulation
- Current View on Green Tea Catechins Formulations, Their Interactions with Selected Drugs, and Prospective Applications for Various Health Conditions
- In silico Structure-based Identification of Novel Acetylcholinesterase Inhibitors Against Alzheimer’s Disease
- Development and In Vitro Evaluation of Controlled Release Viagra® Containing Poloxamer-188 Using Gastroplus™ PBPK Modeling Software for In Vivo Predictions and Pharmacokinetic Assessments
Go deeper in Evidence Hub
Evidence Hub adds a study data sheet (design, sample size, evidence level, key numbers), an AI summary and a chat grounded in the paper's text.