Managing Hypertension and IgG4-Related Disease

CardioNerds nerd out with Drs. Karishma Rahman (Mount Siani Vascular Medicine fellow), Shu Min Lao (Mount Sinai Rheumatology fellow), and Constantine Troupes (Mount Sinai Vascular Surgery fellow). They discuss the following case: A 20-year-old woman with a history of hypertension (HTN), initially thought to be secondary to a mid-aortic syndrome that resolved after aortic stenting, presents with a re-occurrence of HTN. The case will go through the differential diagnosis of early onset HTN focusing on structural etiologies of HTN, including mid-aortic syndrome and aortitis. We will also discuss the multi-modality imaging used for diagnosis and surveillance, indications and types of procedural intervention, and how to diagnose and treat an underlying inflammatory disorder leading to aortitis. The expert commentary was provided by Dr. Daniella Kadian-Dodov, Associate Professor of Medicine and Vascular Medicine specialist at the Icahn School of Medicine at Mount Sinai. Audo editing was performed by Dr. Chelsea Amo-Tweneboah, CardioNerds Academy Intern and medicine resident at Stony Brook University Hospital. US Cardiology Review is now the official journal of CardioNerds! Submit your manuscript here. CardioNerds Case Reports PageCardioNerds Episode PageCardioNerds AcademyCardionerds Healy Honor Roll CardioNerds Journal ClubSubscribe to The Heartbeat Newsletter!Check out CardioNerds SWAG!Become a CardioNerds Patron! Case Media - Hypertension With a Twist Pearls - Hypertension With a Twist Early onset hypertension (HTN) and lower extremity claudication should raise suspicion for aortic stenosis (including mid-aortic syndrome). Initial evaluation should include arterial duplex ultrasound and cross-sectional imaging such as CT or MR angiogram of the chest, abdomen, and pelvis to assess for arterial stenosis involving the aorta and/or branching vessels. Mid-aortic syndrome can have multiple underlying etiologies. Concentric aortic wall thickening should raise suspicion for an underlying inflammatory disorder. Initial evaluation should include inflammatory markers such as ESR, CRP, and IL-6, but normal values do not exclude underlying aortitis.  While Takayasu arteritis is the most common inflammatory disorder associated with mid-aortic syndrome, IgG4-RD should also be a part of the differential diagnosis. IgG subclass panel can detect IgG4-RD with elevated serum IgG4 levels, but some cases can require pathology for diagnosis. Catheter based intervention is a safe and effective treatment of aortic stenosis for both primary aortic stenosis and post-procedural re-stenosis. Multi-modality imaging, including cross-sectional imaging and duplex ultrasound, plays a central role for the diagnosis, management, and post-procedural surveillance of aortic disease. A multi-disciplinary team (as exemplified by the participants of this podcast!) is essential for the management of complex aortopathy cases to optimize clinical outcomes. Show Notes - Hypertension With a Twist 1.  Early onset HTN can have multiple etiologies – aortic stenosis (including but not limited to secondary to congenital aortic coarctation and mid–aortic syndrome, as well as in stent re-stenosis if there is a history of aortic stenting), thrombosis, infection, inflammatory/autoimmune disorders, renovascular disease, polycystic kidney disease, and endocrine disorders. 2. Mid-aortic syndrome is characterized by segmental or diffuse narrowing of the abdominal and/or distal descending aorta with involvement of the branches of the proximal abdominal aorta (renal artery, celiac artery, superior mesenteric artery) and represents approximately 0.5 to 2% of all cases of aortic narrowing. Underlying etiologies include genetic syndromes, inflammatory, non-inflammatory, and idiopathic. It is important to have a high suspicion of underlying inflammatory disorders if cross-sectional i...