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Takayasu Arteritis Presenting As a Pulmonary-Renal Syndrome

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Takayasu Arteritis Presenting As a Pulmonary-Renal Syndrome Takayasu Arteritis Presenting as a Pulmonary-Renal Syndrome BRIAN J. SAVAGE, MD; ROHIT K. GUPTA, MD; JOHN ANGLE, MD; MARK D. OKUSA, MD ABSTRACT: Takayasu arteritis is an uncommon disease transluminal angioplasty of the left renal artery normal- with a variety of presentations. We report a case of ized kidney function. The initial presentation of Taka- Takayasu arteritis with a presentation of a pulmonary- yasu arteritis as a pulmonary-renal syndrome with se- renal syndrome in a 22-year-old woman. She presented vere acute renal failure and diffuse pulmonary in acute respiratory failure with hemoptysis and acute hemorrhage is unusual; to our knowledge, this has not renal failure; interestingly, however, the renal biopsy been described previously in the literature. We provide was normal. Magnetic resonance angiography (MRA) a clinical review of Takayasu arteritis and a discussion of showed significant narrowing in the distal abdominal systemic manifestations pertinent to the case. KEY IN- aorta with bilateral renal and common iliac artery oc- DEXING TERMS: Acute renal failure; Vasculitis; Reno- clusions. Thoracic and abdominal angiogram confirmed vascular hypertension. [Am J Med Sci 2003;325(5): MRA findings of type IV Takayasu arteritis. Percutaneous 275–281.] for a father with hypercholesterolemia who died of myocardial akayasu arteritis is a rare, idiopathic, inflam- infarction at age 58 and a mother with hypothyroidism. Tmatory disease that primarily affects large ves- At a local hospital a physical examination showed the patient sels in young women.1–4 Numerous case reports in to be tachypneic and tachycardic with a temperature of 35.7°C, the literature demonstrate the wide variety of pre- respiratory rate of 20 breaths/min, pulse of 141 beats/min, and sentations, depending on the segment of vessels and blood pressure of 181/113 mm Hg. Rales and wheezes were heard diffusely by chest auscultation. Chest radiograph showed diffuse accompanying organ systems involved. This results bilateral alveolar infiltrates. Arterial blood gas results included a in a disorder that provides a significant diagnostic pH of 7.20, pCO2 of 33 mm Hg, pO2 of 47 mm Hg, oxygen challenge to the clinician. The purpose of this case saturation of 67% on3Lofoxygen by nasal cannula. She was report is to describe an unusual presentation of intubated and ventilated. Bloody secretions were suctioned from Takayasu arteritis initially mimicking a pulmonary- the oral endotracheal tube, believed to be secondary to pulmonary hemorrhage. Other laboratory data are presented in Table 1. She renal syndrome and to provide a clinically relevant was treated with high-dose steroids, transfused with 2 units of review of the disease entity. packed red blood cells for worsening anemia, and transferred to the University of Virginia Health System. Case Report On transfer, chest auscultation demonstrated anterolateral rales. Abdominal exam showed mild midepigastric, periumbilical, A 22 year-old woman with a 1-month history of ankle pain and and right upper quadrant tenderness. Trace ankle edema was edema presented to a local hospital with 2 days of shortness of present and right foot was cool with mottling. Dorsalis pedis breath, midepigastric pain, nausea, and vomiting. History from pulses were diminished bilaterally but present by Doppler tech- the family members noted her to have exhibited extreme fatigue nique. She was oliguric and her renal function continued to over at least the prior month. Past medical history was signifi- decline, with a serum urea nitrogen of 76 mg/dL and creatinine of cant for hyperthyroidism diagnosed 2 years before admission and 6.4 mg/dL. Urinalysis dipstick showed specific gravity of 1.025, iron deficiency anemia diagnosed 2 weeks before admission. Med- pH 5.0, protein 1ϩ, and large blood, leukocyte esterase negative, ication was limited to propylthiouracil for treatment of hyperthy- and nitrite negative. Urine microscopy showed many red blood roidism. She did not smoke tobacco or drink alcohol. She was cells, many granular casts, few hyaline casts, no cellular casts, employed as a telephone operator. Family history was significant and a few calcium oxalate crystals per high-power field. This urine specimen most probably represented acute tubular necro- sis, but a glomerular process could not be excluded. High-dose steroids were continued, and plasmapheresis and From the Division of Nephrology, Department of Medicine (BJS, hemodialysis were initiated for presumed pulmonary-renal RKG, MDO), and Department of Radiology (JA), University of Vir- syndrome secondary to a small vessel vasculitis. Renal biopsy ginia, Charlottesville, Virginia. was performed and sent for light microscopy, immunofluores- Submitted July 31, 2002; accepted December 12, 2002. cence, and electron microscopy. On light microscopy, a total of This case was presented at the Annual Meeting of the National 18 glomeruli revealed only a slight increase in cellularity and Kidney Foundation’s annual meeting, April 17–22, 2000, in At- no inflammation of medium-sized vessels. On immunofluores- lanta, Georgia. cence, the specimen had no significant staining, and on elec- Correspondence: Mark D. Okusa, M.D., Division of Nephrology, tron microscopy, it did not contain electron dense deposits, Box 800133, University of Virginia Health System, Charlottes- indicating therefore a normal renal biopsy. Anti-neutrophil ville, VA 22908 (E-mail: [email protected]). cytoplasmic antibody was present in a perinuclear pattern at a THE AMERICAN JOURNAL OF THE MEDICAL SCIENCES 275 Takayasu Arteritis Presenting as a Pulmonary-Renal Syndrome Table 1. Initial Laboratory Data Magnetic resonance angiography (MRA) demonstrated a long segment of narrowing in the distal abdominal aorta with Serum bilateral renal and common iliac artery occlusions (Figure 1A). White blood cells 2.8 103/␮L Collateral flow reconstituted the distal renal and external iliac Hemoglobin 9.5 g/dL arteries. Thoracic (Figure 1B) and abdominal contrast angio- Platelets 186 103/␮L gram (Figure 1C) confirmed MRA findings of type IV Takayasu Sedimentation rate 75 mm/hour arteritis (Hata classification).1 The left renal artery was di- Sodium 134 mmol/L lated to 5 mm with percutaneous transluminal angioplasty. Potassium 3.9 mmol/L The right renal artery was completely occluded. Urine output Chloride 96 mmol/L improved immediately and serum creatinine fell to 1.7 by Bicarbonate 22 mmol/L hospital day 10. Bronchoscopy demonstrated bloody secretions Urea nitrogen 45 mg/dL without obvious source, and the patient was extubated. A Creatinine 3.5 mg/dL two-dimensional echocardiogram revealed a severely de- Glucose 135 mg/dL creased ejection fraction with normal left ventricle wall thick- Calcium 9.3 mg/dL ness. Cardiac catheterization confirmed no pulmonary hyper- Albumin 2.8 mg/dL Prothrombin 13.9 seconds tension or coronary artery disease. An endocardial biopsy and International normalized ratio 1.3 histological analysis did not demonstrate myocarditis. MRA of Urinalysis the pulmonary vessels did not show evidence of vasculitis. She Specific gravity 1.025 was discharged on hospital day 20 with a serum creatinine of Protein 100 mg/dL 0.9 mg/dL and was maintained on 60 mg of prednisone once Blood Small daily and lisinopril, amlodipine, and furosemide. Urine fractional excretion of sodium 0.6% She was seen in follow-up 10 months after discharge com- plaining of bilateral lower extremity paresthesias that wors- ened with exercise. Her serum urea nitrogen was 11 mg/dL and creatinine was 0.9 mg/dL. Her blood pressure was 160/94 on titer Ͼ1:160. The test for c–anti-neutrophil cytoplasmic auto- 12.5 mg of prednisone every other day, 10 mg of amlodipine antibodies was negative. Other negative serologic studies in- once daily, 50 mg of losartan twice daily, 81 mg of aspirin once cluded anti-glomerular basement membrane antibody, anti- daily, and oral contraceptive pills. An angiogram showed oc- nuclear antibody, cryoglobulins, anti-human immuno- clusions of the bilateral distal iliac arteries, reconstituting deficiency virus, and hepatitis screen. The erythrocyte sedi- distally. She underwent successful aortobifemoral bypass mentation rate was normal at 20 mm/hour. grafting. Figure 1. (A) MRA demonstrating narrowing of the distal abdominal aorta with bilateral renal and common iliac artery occlusion. (B) Angiogram of thoracic aorta. (C) Angiogram of abdominal aorta. 276 May 2003 Volume 325 Number 5 Savage et al Discussion Table 2. Clinical Features of Takayasu Arteritis Epidemiology. Takayasu arteritis is a rare, in- %at flammatory, idiopathic disease of the aorta and its Presentation or major branches that primarily affects young women. Clinical Features during Course The disease is chronic, with the gradual develop- 1. Vascular ment of stenoses that may lead to vascular occlu- a. Diminished or absent pulse 60–98 sions. Subsequent organ and/or limb ischemia pro- b. Carotid bruit 50–70 duces a variety of clinical presentations and c. Claudication 9–70 manifestations reflecting inadequate perfusion. The d. High blood pressure 33–59 e. Subclavian 22–55 nonspecific and subacute nature of the signs and f. Carotodynia 32 symptoms combined with the low prevalence of the g. Abdominal 10–24 disease usually results in a delay in diagnosis. The h. Femoral bruit 5 estimated incidence derived from a retrospective 2. Central nervous system a. Lightheadedness, dizzy 33–41 Mayo Clinic study of 32 cases was 2.6 cases per b. Visual abberation 9–24 2 million persons per year. The number was based on c. Visual loss 8 a predominantly white population in Olmstead d. Stroke 8 County, Minnesota. The median time to diagnosis e. Transient ischemic attack 8 was 18 months in 1 study2 and 10 months in anoth- 3. Musculoskeletal a. Chest wall 30 3 er. The median age at diagnosis was 31 years b. Joint pain 30 (range, 15–48 years).2 Women under 20 are fre- c. Myalgia 12 quently involved. Mortality rate is 3 to 6% at ~5 4. Constitutional years from diagnosis. The 2 largest North American a.
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