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Takayasu's arteritis associated with crescentic glomerulonephritis.

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45 1
BRIEF REPORT
TAKAYASU’S ARTERITIS ASSOCIATED WITH CRESCENTIC
GLOMERULONEPHRITIS
DAVID B. HELLMANN, KENNETH HARDY, STAN LINDENFELD, and ERNEST RING
We describe the initial course and followup of a
16-year-old white female patient who presented in 1983
with aortitis and rapidly progressive renal failure. Renal
biopsy revealed crescentic glomerulonephritis. Segmental occlusions of the distal aorta and both common iliac
arteries were demonstrated on aortography. Initially,
her renal function and arterial occlusions markedly
improved with pulse prednisolone therapy, but 34
months later, the glomerulonephritis and aortitis worsened. This case report, the first of its kind, documents
both the association of Takayasu’s arteritis with crescentic glomerulonephritis and a marked, though incomplete, response to pulse therapy.
Takayasu’s arteritis is a vasculitis of unknown
cause that affects the aorta and branch vessels, chiefly
in young women. Glomerular involvement is rare and
largely exists as a mild mesangial proliferative glomerulonephritis (1-3). We report the first case of Takayasu’s arteritis known to be associated with rapidly
progressive renal disease due to crescentic glomerulonephritis. Pulse prednisolone therapy produced a
From The Johns Hopkins Hospital, Baltimore, Maryland;
The Howard Hughes Medical Institute and Baylor College of
Medicine, Houston, Texas; and The University of California, San
Francisco.
David B. Hellmann, MD: Assistant Professor of Medicine,
The Johns Hopkins Hospital; Kenneth Hardy, MD, PhD: Assistant
Investigator, Howard Hughes Medical Institute and Assistant Professor of Medicine and Microbiology/Immunology, Baylor College
of Medicine; Stan Lindenfeld, MD: Associate Clinical Professor of
Medicine, The University of California, San Francisco; Ernest Ring,
MD: Professor of Radiology, The University of California, San
Francisco.
Address reprint requests to David B. Hellmann, MD, 1012
Blalock, The Johns Hopkins Hospital, Baltimore, MD 21210.
in revised
Submitted for DublicationMav 16. 1986:
form September 15, 1686.
Arthritis and Rheumatism, Vol. 30, No. 4 (April 1987)
marked improvement in our patient’s renal function,
and improvement in the aortitis was angiographically
documented.
Case report. The patient, a 16-year-old nonAsian white woman, was admitted on March 25, 1983
to the University of California’s Moffitt Hospital, San
Francisco, for evaluation of aortitis. Eight months
before admission, she had developed bilateral
claudication, chills, nausea, and weight loss. Hematuria was noted during the month before admission.
The patient denied ocular symptoms, Raynaud’s phenomenon, abdominal pain, joint pain, or erythema of
the external ear or nasal bridge. The blood pressure
was 130/70 mm Hg in her right arm and 126/70 in the
left. A vascular surgeon noted that the carotid,
subclavian, radial, and aortic pulses were 4+, whereas
the femoral, popliteal, and dorsal pedal pulses were
2+ bilaterally. Bruits were present over both femoral
arteries and the left flank. An ophthalmologist noted
no retinal abnormalities. The serum creatinine level
was 3.9 mg/dl and the blood urea nitrogen level was 45
mg/dl. Urinalysis revealed red blood cell casts. A
24-hour urine sample contained 736 mg of protein. The
creatinine clearance rate was 17 cc/minute/l.73 mm’.
The hematocrit was 18.8%. The Westergren erythrocyte sedimentation rate (ESR) was 135 mdhour. The
following test results were normal or negative: stool
guaiac, electrocardiogram, antistreptolysin 0, rheumatoid factor, antinuclear antibody, rapid plasma
reagin, and total hemolytic complement. An aortogram showed segmental occlusion at the terminal aorta and at both iliac arteries. There were numerous
collateral vessels to the lower extremities via the
lumbar arteries (Figure IA). The left renal artery was
BRIEF REPORTS
452
A
B
Figure 1. Initial and followup aortograms of a young woman who was diagnosed as having Takayasu's arteritis associated with crescentic
glomerulonephritis.A, A right transfemoral aortogram taken shortly after disease onset, demonstrating segmental occlusion of the distal aorta
and both common iliac arteries. Large lumbar arterial collaterals are present. B, An intravenous digital subtraction aortogram that was
performed 18 months later, showing a patent, but slightly narrowed, distal aorta, and widely patent right common and external iliac arteries.
The left common iliac artery remains occluded.
stenosed, and the left intrarenal arterial branches were
pruned and irregular.
On the basis of the patient's clinical course,
deterioration of renal function, and active urinary
sediment, a tentative diagnosis of rapidly progressive
glomerulonephritis was made. Consequently, on
March 26, 1983 the patient was begun on a regimen of
pulse steroid therapy (prednisolone, 1 gm intravenously, daily for 3 days; followed by oral prednisone,
50 mg daily). On March 30,1983 the patient underwent
a percutaneous left renal biopsy. Light microscopy
showed that 7 of 8 glomeruli contained areas of segmental fibrinoid necrosis of capillary tufts associated
with crescents (Figure 2). Two of the crescents were
epithelial and 5 were fibroepithelial. There was no
evidence of vasculitis. The crescents stained for fibrin,
but not for immunoglobulin or complement. The specimen for electron microscopy included 6 glomeruli, 5
of which had crescents. No electron-dense immune
deposits were present in mesangial areas or in periph-
eral capillary walls. The pathologic diagnosis was
diffuse segmental necrotizing and crescentic glomerulonephritis, consistent with rapidly progressive glomerulonephritis, idiopathic type.
Following institution of steroid therapy, the
patient's serum creatinine level peaked at 4.5 mg/dl,
then fell to 2.5 mg/dl by April 3, 1983. The prednisone
was tapered, and was stopped by September 1984. On
October 29, 1984, an intravenous, digital aortogram
demonstrated that the right common iliac artery was
patent over its entire course (Figure 1B). This was a
marked improvement over the initial findings. The left
common iliac artery was occluded, but numerous
collateral vessels were present. Reevaluation on May
10, 1985 showed her blood pressure to be 125170 mm
Hg, the serum creatinine level was 1.5 mg/dl, the
creatinine clearance rate was 50 cdminute, and the
24-hour urine protein level was 500 mg. The proximal
and distal pulses in her right leg were normal. The left
453
BRIEF REPORTS
Figure 2. Photomicrograph of representative glomerulus, showing segmental necrosis and epithelial crescent in early stages of
organization (hematoxylin and eosin stained, original magnification X 550.)
femoral pulse was 1+, but no distal pulses were
palpable.
In January 1986, the patient developed epistaxis
and headaches. Evaluation in March 1986 showed that
the blood pressure was 150/100 mm Hg in both arms,
and there was no evidence of peripheral ischemia. The
serum creatinine level was 2.1 mg/dl, the creatinine
clearance rate was 38 cc/minute/l.73 mm2, the 24-hour
urine protein level was 3,992 mg, and the ESR was 92
mmhour. Angiography showed slight worsening of the
intrarenal aortic stenosis. The left renal artery was
then totally patent, and the iliac vessels were unchanged. Renal biopsy showed a focal necrotizing
glomerulonephritis with interstitial inflammation; 50%
of the glomeruli were sclerosed. In view of the active
necrotizing glomerulonephritis, pulse prednisolone (1
gm intravenously, daily for 3 days), followed by oral
prednisone every other day, was administered. One
month later, the ESR was 30 mmhour, and the serum
creatinine level was 1.8 mg/dl.
Discussion. This is the first description of
Takayasu’s arteritis associated with crescentic glomerulonephritis. The diagnosis of Takayasu’s arteritis in
this young woman was suggested by claudication with
diminished pulses, and was confirmed by the findings
of the aortogram. The patient’s chills, nausea, weight
loss, and elevated ESR represented early manifestations of the aortitis. Her vasculitis was limited to the
lower abdominal aorta (type 111). She did not have
aortic arch (type I), panaortic (type 11), or pulmonary
artery (type IV) involvement (4). Restriction of the
disease to the thoracic aorta or, as in this case, to the
abdominal aorta, is not uncommon in patients younger
than 20 years (2). Other disorders that can involve the
aorta, such as syphilis, coarctation, cystic medical
necrosis, and relapsing polychondritis, had been
excluded.
454
Though crescentic glomerulonephritis has been
associated with a wide variety of disorders (including
Wegener’s granulomatosis, systemic lupus erythematosus, Goodpasture’s syndrome, diabetes, poststreptococcal glomerulonephritis, and polyarteritis
nodosa), it has not been previously reported to occur
with Takayasu’s arteritis. Indeed, renal manifestations
of Takayasu’s arteritis are uncommon and are usually
confined to complications due to ischemia (4-9).
Glomerular disease has, however, been reported in 8
other patients with Takayasu’s arteritis (1-4). In all of
these patients, the renal lesions were limited to a mild
mesangial proliferative or focal glomerulonephritis
( 1 4 ) . In contrast, our patient had rapidly progressive
renal failure caused by crescentic glomerulonephritis.
This case is instructive in that it documents
both the association of Takayasu’s arteritis with crescentic glomerulonephritis and a gratifying, though
incomplete, response to pulse steroid therapy. Following initial therapy, the patient’s systemic symptoms
resolved, and her renal function had nearly normalized. Serial angiograms over the first 2 years after
treatment showed improvements in some of the affected vessels, most strikingly in the right iliac artery.
Though there are other reports which suggest that
glucocorticoids can improve the signs and symptoms
of Takayasu’s arteritis, few of these studies provide
angiographic proof of vascular improvement (6,lO-12).
Nearly 3 years after the initial pulse prednisolone treatment, the patient’s glomerulonephritis and
aortitis recurred. Treatment with pulse prednisolone
was repeated and appears to be effective. Although the
optimal treatment for Takayasu’s arteritis has not been
established, most authors have reported the use of
moderate doses of prednisone (30-40 mg/day).
Cytotoxic agents have apparently successfully suppressed the arteritis in patients whose disease worsens
with prednisone treatment (12). Our initial choice of
pulse prednisone for this patient was based on the
favorable results achieved with this regimen in idiopathic crescentic glomerulonephritis (13,14). The recurrence of the aortitis and renal disease emphasizes
that the pulse prednisone suppressed the disease, but
did not achieve a long-term remission. It is not known
whether cyclophosphamide therapy, which has been
effective for crescentic glomerulonephritis due to
Wegener’s granulomatosis (19, would also be effective for treating crescentic glomer ulonephritis associated with Takayasu’s arteritis.
BRIEF REPORTS
Acknowledgment. We thank Dr. Claude Biava for
providing the photomicrograph of the renal biopsy.
REFERENCES
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10. Gardner JD, Lee KR, Abdou NI: Takayasu’s arteritis:
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11. Fraga A, Mintz G, Valle L , Flores-Izquierdo G: Takayasu’s arteritis: frequency of systemic manifestations
(study of 22 patients) and favorable response to maintenance steroid therapy with adrenocorticosteroids (12
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12. Shelhamer JH, Volkman DJ, Parillo JE, Lawley TJ,
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13. Bolton WK, Couser WG: Intravenous pulse methylprednisolone therapy of acute crescentic rapidly progressive
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