Ernste, Floranne C.

This present case pertains to a 48-year-old woman with a history of antiphospholipid syndrome, who presented with progressive fatigue, generalized weakness, and orthopnea acutely. She had a prior diagnosis of antiphospholipid syndrome with recurrent deep vein thromboses (DVTs) and repeated demonstration of lupus anticoagulants. She presented in cardiogenic shock with markedly elevated troponin and global myocardial dysfunction on echocardiography, and cardiac catheterization revealed minimal disease. Cardiac magnetic resonance imaging was performed, which revealed findings of perfusion defects and microvascular obstruction, consistent with the pathophysiology of catastrophic antiphospholipid syndrome (CAPS). Diagnosis was made based on supportive imaging, including head magnetic resonance imaging (MRI) revealing multifocal, acute strokes; microvascular thrombosis in the dermis; and subacute renal infarctions. The patient was anticoagulated with intravenous unfractionated heparin and received high-dose methylprednisolone, plasmapheresis, intravenous immunoglobulin, and one dose each of rituximab and cyclophosphamide. She convalesced with eventual myocardial recovery after a complicated course. The diagnosis of CAPS relies on the presence of (1) antiphospholipid antibodies and (2) involvement of multiple organs in a microangiopathic thrombotic process with a close temporal association. The myocardium is frequently affected, and heart failure, either as the presenting symptom or cause of death, is common. Despite echocardiographic evidence of myocardial dysfunction in such patients, MRIs of CAPS have not previously been reported. This case highlights the utility in assessing the involvement of the myocardium by the microangiopathic process with MRI. Because the diagnosis of CAPS requires involvement in multiple organ systems, cardiac MRI is likely an underused tool that not only reaffirms the pathophysiology of CAPS, but could also clue clinicians in to the possibility of a diffuse thrombotic process.

ObjectiveThe role of cardiovascular disease (CVD) risk factors in psoriatic arthritis (PsA) is poorly understood. We examined the prevalence of CVD risk factors at initial onset of PsA and compared the observed incidence of CVD events with that predicted by the Framingham Risk Score (FRS) to determine its applicability in this patient population.MethodsA population‐based incidence cohort of 158 patients with PsA who fulfilled Classification of Psoriatic Arthritis criteria for PsA in 1989–2008 was assembled. Medical records were reviewed to ascertain CVD risk factors and CVD events. Future risk of CVD was estimated using the FRS algorithm.ResultsMean age was 43.4 years (range 19–74 years), 61% were men, and 44% were obese (body mass index ≥30 kg/m2). Fifty‐four patients (34%) presented with ≥2 CVD risk factors at PsA incidence. Among 126 patients ages ≥30 years at PsA incidence with no prior history of CVD, 33% had an FRS ≥10%, with 11% having an FRS ≥20%, and 18 experienced a CVD event in the first 10 years of disease duration. The 10‐year cumulative incidence of CVD events was 17% (95% confidence interval [95% CI] 10%–24%), almost twice as high as the predicted incidence based on the FRS (standardized incidence ratio 1.80, 95% CI 1.14–2.86; P = 0.012).ConclusionThe majority of newly diagnosed PsA patients have a >10% risk of CVD within 10 years of PsA incidence. The CVD risk in these patients is higher than expected and underestimated by the FRS.