A 46‐year‐old woman presented to the referring hospital with uncontrolled high blood pressure for the preceding several weeks. Two months prior, she underwent an outpatient ablation for supraventricular tachycardia. At that time, she was prescribed a new antihypertensive regimen and prednisone for interstitial lung disease. Past medical history was notable for scleroderma with diffuse skin involvement. At the referring hospital, her creatinine was 4.mg/dL, an increase from 1.4 mg/dL at one month prior. Schistocytes were found on peripheral blood smear. With concern for scleroderma renal crisis (SRC) versus thrombotic thrombocytopenic purpura (TTP), she was started on captopril and clonidine. Despite blood pressure improvement, she developed worsening thrombocytopenia and progressive renal failure. She was transferred to our hospital for further evaluation. On arrival, her blood pressure was 196/88. She had diffusely thickened and tightened skin with vitiliginous patches on the face, chest, back, and upper extremities. Pertinent laboratory findings included: BUN 110 mg/dL, creatinine 7 mg/dL, platelets 110,000/μL, and hemoglobin 10.6 g/dL. Urine examination showed mild proteinuria but no active sediment. A diagnosis of SRC was made and captopril was rapidly titrated up. The time from onset of hypertension to treatment with maximum captopril dose was roughly three months. Her blood pressure eventually stabilized at 120s/80s, but her renal function never recovered. She was discharged on an outpatient dialysis regimen and low‐dose ACE‐inhibitor treatment.
Uncontrolled hypertension is a common problem encountered by hospitalists. Given the ubiquitous nature of the diagnosis, it is critical for physicians to be able to identify a true hypertensive crisis, such as SRC. SRC occurs in 10‐15% of patients with the diffuse form of cutaneous systemic sclerosis. Risk factors include recent corticosteroid use, early and diffuse skin involvement, and presence of auto‐antibodies to ribonucleic acid polymerase. SRC typically presents with abrupt onset of accelerated hypertension and progressive renal failure. Other clinical features may include microangiopathic hemolytic anemia and nonnephrotic proteinuria. SRC shares some clinical characteristics with both uncomplicated hypertension and TTP, but delay in making an accurate diagnosis and initiating treatment promptly may result in devastating consequences. In this patient, diagnosis and treatment were significantly delayed. Prior to the use of ACE‐inhibitors, the current standard of care, SRC had an almost invariably fatal outcome. Although ACE‐inhibitors have dramatically improved mortality, 20‐50% of patients progress to end‐stage renal disease.
This case illustrates the importance of considering SRC when evaluating hypertension in the hospitalized patient. Failure to recognize and treat promptly with ACE‐inhibitors can lead to renal failure and death.
To cite this abstract:Zuo R, Sharma P. Hypertension: Going More Than Skin Deep. Abstract published at Hospital Medicine 2014, March 24-27, Las Vegas, Nev. Abstract 691. https://www.shmabstracts.com/abstract/hypertension-going-more-than-skin-deep/. Accessed February 16, 2019.