1999 Resident Poster Competition
Karan D. Singh M.D.
Univ. of Missouri--Columbia
A 73 Years Old Man With a Rash, Rapidly Progressive Renal Failure and Abrupt Onset of Dyspnea
A 73 year old man was seen in general medicine clinic for a progressive rash on both lower extremities involving the extensor surface of knees, dorsum of feet and the toes. The rash was thought to be livedo reticularis. On routine laboratory evaluation, the blood urea nitrogen and serum creatinine concentrations were found to be 69 milligrams per dl and 5.7 milligrams per dl respectively. During the six months prior, the creatinine concentration had ranged between 1.0 and 1.4 milligrams per dl. He was admitted for the evaluation of rapidly progressive renal failure with the rash. He had a long-standing history of hypertension, hyperlipidemia dn chronic obstructive pulmonary disease. He was on treatment with an ACE inhibitor, a statin, inhaled beta-2 agonists and steroids. He had smoked one and a half packs per day of cigarettes for 50 years. Physical examination revealed a pale, averagely built man, Temperature was 3.69 degrees Celsius, pulse was 88, respiratory rate was 20 per minute, blood pressure was 146/80. JVP was 6cm above angle of louis. Inspiratory crackles were heard over the lower third of both lungs. A grade 2/6 ejection systolic murmur was maximal at the aortic area. No S3 or S4 was heard. There was no peripheral edema. Femoral pulses were 2+ and dorsalis pedis were 1+.
A proteinuria of one gram per day was noted. Urine sediment contained two WBCs and six RBCs per high-power field. Serum protein electrophoresis and urine protein electrophoresis were normal. A test for Bence Jones proteins was negative. Ultrasonographic examination of the kidneys was normal. CT scan of the abdomen revealed a 4.9cm infrarenal abdominal aortic aneurysm. An echocardiogram revealed left ventricle hypertrophy, normal left ventricular ejection fraction, mild mitral regurgitation, left atrial enlargement and aortic sclerosis. A recent lipid profile had revealed a cholesterol concentration of 216 milligrams per dl and a triglyceride concentration of 235 milligrams per dl. Other lab results were unremarkable. An electrocardiogram was unremarkable. X-ray of the chest was consistent with emphysema and bilateral small pleural effusions, Hepatitis serologies were negative, serum cryoglobulins were negative, and a test for anti-neutrophilic cytoplasmic antibody was negative.
While in the hospital, he became progressively dyspneic. There was no orthopnea. Repeat Chest x-ray showed bilateral scattered infiltrates. He was afebrile with no cough or sputum production. Renal function continued to deteriorate. Arterial blood gases revealed a p02 of 45 pC02 of 40 pH of 7.43 on room air. A procedure yielding the diagnosis was performed.
Over the ensuing days, Renal and respiratory function continued to deteriorate. Hemodialysis was instituted. He was also treated with IV Methylprednisone, empiric antibiotics and bronchodilators. None of the therapies halted the course of the disease. Patient and family declined intubation and mechanical ventilation. He died on hospital day 28. Autopsy was performed.
Discussion: A differential diagnosis of bacterial endocarditis, cryoglobulinemia, antiphospholipid antibody syndrome, pulmonary-renal syndromes, Renal artery stenosis, cholesterol embolic disease, multiple myeloma, amyloidosis, SLE and other collagen vascular diseases was considered. Skin and open kidney biopsy together clinched the diagnosis of cholesterol embolic disease. Transesophageal echocardiogram revealed multiple large sessile as well as polypoidal atheromatous lesions. This disease entity clearly occurs more commonly than is generally appreciated. Although vascular intervention, thrombolytics or anticoagulants precipitate a substantial proportion of cases, 40-60 percent occur spontaneously. The syndrome essentially remains untreatable although anecdotal reports suggest improvement with statins. Anti-coagulation is contraindicated. The cause of dyspnea in these patients had remained enigmatic. Extensive pulmonary cholesterol emboli have been demonstrated.
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