SESSION TITLE: ICU Cases
SESSION TYPE: Affiliate Case Report Slide
PRESENTED ON: Sunday, October 27, 2013 at 01:15 PM - 02:45 PM
INTRODUCTION: The clinical manifestation of Catastrophic Antiphospholipid Syndrome (aka Asherman’s syndrome) vary and challenging to diagnose due to acute onset of thrombosis resulting in multi-organ failure.
CASE PRESENTATION: A healthy sixteen year-old female patient presented with acute kidney failure, hypoxia, and vision changes. Several weeks prior, she had right-sided flank pain. At the local ED, an abdominal ultrasound was normal, so was diagnosed with constipation. The pain resolved on its own. During the following week, she developed a dry cough, odynophagia, malaise, fatigue, and painless blurry vision with erythema in her right eye. She was diagnosed with a viral URI at her local clinic. The following day, blurry vision progressed to involve both eyes. Returning to the ED, she was found to be diaphoretic, tachycardic to 150's bpm, and hypertensive to 180-200's/130's mmHg. She subsequently developed bradycardia and hypoxia to 70's requiring urgent intubation. Chest x-ray showed diffuse bilateral opacities. Labs revealed a Creatinine of 5 mg/dL. Echocardiogram indicated depressed EF of 30%. CT angiogram confirmed subacute to chronic thrombotic occlusion of the abdominal aorta from the superior mesenteric artery to the bifurcation of the renal arteries. Percutaneous biopsy of the kidney showed thrombotic microangiopathy, severe interstitial fibrosis, and tubular atrophy. This led to serology testing, which showed ANA titer 1:120 and the presence of anti-double stranded DNA and anti-phospholipid antibodies.
DISCUSSION: The rapid development of Stage III Acute Kidney Failure was secondary to decreased perfusion to kidneys, which likely developed over the course of several days to weeks. The CT angiogram showing extensive thrombosis of her aorta and kidney biopsy helped narrow the differential diagnosis, including Takayasu's arteritis (type III or IV), polyarteritis nodosa, TTP-HUS, and DIC. However, ultimately the diagnosis was SLE with Catastrophic Antiphospholipid Syndrome.
CONCLUSIONS: Thrombotic microangiopathy (TMA) is characterized by fibrin thrombi in glomeruli and/or arterioles and has been the most frequently reported intrarenal vascular lesion in aPL-positive patients with or without lupus. In patients with these lesions, especially in those with SLE, aPL antibody testing is recommended.
Reference #1: Cervera, R., et al., Task Force on Catastrophic Antiphospholipid Syndrome (APS) and Non-criteria APS Manifestations (I): catastrophic APS, APS nephropathy and heart valve lesions. Lupus, 2011; 20(2):165-73
Reference #2: Sciascia, S., et al., Catastrophic antiphospholipid syndrome (CAPS). Best practice & research. Clinical rheumatology, 2012; 26(4):535-41
Reference #3: Tektonidou, M.G., et al., Antiphospholipid syndrome nephropathy in patients with systemic lupus erythematosus and antiphospholipid antibodies: prevalence, clinical associations, and long-term outcome. Arthritis and rheumatism, 2004; 50(8):2569-79
DISCLOSURE: The following authors have nothing to disclose: Sarah Lee
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