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Retroperitoneal fibrosis associated with aortitis.

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Retroperitoneal fibrosis associated with aortitis
Retroperitoneal fibrosis (RPF) was established by
Ormond as a clinical entity (1); however, the mechanism that
accounts for fibrosis in the retroperitoneal space remains
unclear. Patients with RPF usually demonstrate obstruction
of the ureters, resulting in hydronephrosis, but they frequently also have general symptoms such as weight loss and
malaise. There have been several reports of RPF in overlap
with systemic autoimmune diseases, such as systemic lupus
erythematosus (2), Raynaud’s disease with pulmonary fibrosis (3), and systemic vasculitis (4). Thus, certain systemic
pathogenetic processes may contribute to the development
of the disease.
We report here the case of a patient with RPF
associated with aortitis, which suggests that part of RPF is
the local expression of a vasculitis syndrome with circulating
immune complexes.
The patient, a 68-year-old Japanese man, was admitted to our hospital on July 27, 1990 because of weight loss,
malaise, and lower back pain. He had a 10-year history of
Parkinson’s disease, and had been taking propranolol for 10
years and bromocriptine for 4 years. Physical examination
showed signs of left pleural effusion, a bruit over the left
upper abdomen, and lymphedema of the lower extremities.
The erythrocyte sedimentation rate (ESR) was I15 mmhour,
the hemoglobin level was 8.9 g d d l , and the urinalysis
findings were normal. The C-reactive protein (CRP) level
was 11.65 mg/dl (normal 0 . 1 5 ) , the serum IgG concentration was 3.6 g d d l , but serum complement levels were
normal. Creatinine clearance was decreased to 50 ml/minute.
Serologic studies disclosed positive results for antinuclear antibodies (titer 1:80, with a speckled pattern, on
HEp-2 cell substrate: normal not detected at a 1:40 dilution)
and anti-smooth muscle antibodies (titer 1:40), but negative
results for lupus erythematosus cells, anti-double-stranded
DNA antibodies, and rheumatoid factor. Circulating immune
complexes were increased to 61.6 pg/ml (by anti-Clq binding
assay: normal 0-37.5) and mixed-type cryoglobulins, consisting of IgG at 5.5 mg/dl and IgM at 1.0 mg/dl, were high.
A test for hepatitis B virus antigen gave negative results, and
no significant antibody against viral antigens was detected in
his serum. Chest radiography showed enlargement of the
heart and a left pleural effusion. Echocardiogram confirmed
a large amount of pericardial effusion and pericardiocentesis
demonstrated a bloody exudate.
Computed tomography (CT) scan demonstrated a
periaortic retroperitoneal mass that was markedly enhanced,
and thickening of the aortic wall (Figure IA). RPF was
diagnosed, and propranolol and bromocriptine therapy was
discontinued. The patient’s creatinine clearance rate continued to deteriorate, dropping to 8 muminute on August 10.
Ultrasound examination disclosed hydronephrosis of both
kidneys and a decreased blood flow in the left renal artery.
Double-J stents were inserted in both ureters, and treatment
with prednisolone, 30 mg/day, was initiated. His creatinine
clearance rate recovered to 50 muminute within a few days.
On August 17, intraarterial digital subtraction angiography was performed. There was stenosis and dilatation of
Arthritis and Rheumatism, Vol. 35, No. 10 (October 1992)
Figure 1. A, Computed tomography (CT) scan of the patient’s
abdomen at the level of LS prior to treatment, showing a penaortic
retroperitoneal mass and thickening of the aortic wall. There is
marked enhancement of the mass by the contrast medium. B, CT
scan of the patient’s abdomen at the level of LS (without contrast
medium) after 10 months of prednisolone and azathioprine therapy,
showing a marked reduction in the size of the mass.
the aortic arch, left renal artery, and splenic artery, indicative of aortitis (Figure 2).
A few weeks later, the patient became asymptomatic, and his creatinine clearance rate recovered to 70
ml/minute. The ESR and CRP values decreased to the
normal range, and circulating immune complexes and mixedtype cryoglobulin levels fell below detectable levels. The
pleural effusion disappeared and the pericardial effusion
decreased markedly.
The prednisolone dosage was tapered gradually, but
when decreased to a dosage of I5 mg/day (8 weeks after
initiating the prednisolone therapy), the ESR and CRP
values increased, and an echocardiogram showed an increase in the pericardial effusion. Azathioprine, 50 mg/day,
was initiated, and after this episode, his clinical state became
stable and has remained stable for 1 year. After 10 months of
treatment with prednisolone and azathioprine, a CT scan
revealed dramatic reduction of the retroperitoneal mass
(Figure IB), and an ultrasound examination showed improvement in blood flow in the left renal artery.
Figure 2. Intraarterial digital subtraction angiography of the patient's aorta and its branches. A, Stenosis and dilatation of the aortic
arch (arrow) are demonstrated; the carotid and subclavian arteries
are intact. B, The stem of the left renal artery (arrow) is similarly
affected. These features indicate the presence of aortitis.
This case demonstrates many of the typical features
of RPF. Certain drugs, including propranolol (5) and bromocriptine (6). have been implicated in the etiology of RPF,
and may have contributed to the pathogenesis of RPF in this
patient. In addition, the coexistence of aortitis, pericardial
and pleural effusions, and improvement on treatment with
prednisolone and azathioprine suggest a systemic inflammatory process.
Vasculitis in various organs and different size arteries has been described in patients with RPF (7). However,
arteritis in the aorta and its branches has rarely been
reported. Hardmeier and Hedinger (8) reported a case of
RPF in a subject in whom postmortem examination demonstrated aortitis of the aortic arch, coronary artery, superior
mesenteric artery, and common iliac artery. Jones and
Alexander (9) and Mitchinson (10) also described autopsy
demonstration of RPF and aortitis in 3 subjects. Our patient
represents the first case of RPF associated with aortitis
proved while the subject is living.
Takayasu arteritis typically involves the subclavian
and carotid arteries ( I I ) , but in all 4 reported cases of RPF
associated with aortitis, none of these arteries was affected
(8-10). However, 3 of the 4 subjects with RPF had coronary
artery diseases that are rare complications of Takayasu
arteritis ( I I ) , and 2 of them died of cardiac ischemia (8,9).
Because of differences in the distribution of the aortitis,
typical features of Takayasu arteritis, such as pulselessness
and dizziness, may be lacking in patients with RPF associated with aortitis. Postmortem findings in subjects with RPF
have included massive periaortic and periarterial fibrosis and
atheromatous debris in the inflamed adventitia of the aorta
(10,12). This suggests that aortitis more frequently accompanies the acute phase of RPF than has previously been
Circulating immune complexes and mixed-type cryoglobulins were detected in our patient's serum. Systemic
vasculitis (7), systemic lupus erythematosus ( 2 ) , and glomerulonephritis ( 1 3), which have been linked to circulating
immune complexes, have been reported in patients with
RPF. Biopsy-proven vasculitis at an incidence of -7% (7)
has been demonstrated in patients with RPF, but this may
represent only part of the pathology of this condition.
Pathologic studies of a retroperitoneal mass in a patient with
RPF in an early phase revealed arteritis and periarteritis,
with giant cells and infiltration (14). The size of the retroperitoneal mass in our patient was dramatically reduced after
treatment with prednisolone and azathioprine, and there was
improvement in the aortitis.
This evidence indicates that the fibrous mechanism
of RPF may be linked to vasculitis with circulating immune
complexes. In addition, RPF with vasculitis can involve
almost any organ system and any category of vasculitis, for
example, necrotizing vasculitis (4,7), granulomatous vasculitis (IS), and aortitis. This suggests that RPF is a local
manifestation of a vasculitis syndrome.
If RPF results from a primary vasculitic process, it
would follow that treatment with corticosteroids and immunosuppressants would be effective. Thus, although RPF has
not been classified into any disease category, some patients
with RPF may have an illness which represents a vasculitis
Masataka Kuwana, MD
Shu Wakino, MD
Tadashi Yoshida, MD
Mitsuo Homma, MD
Keio University School of Medicine
Tokyo, Japan
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associates, retroperitoneal, fibrosis, aortitis
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