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Systemic lupus erythematosus in a patient with reiter's syndrome.

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Patients with Reiter’s syndrome typically exhibit the triad of urethritis, conjunctivitis, and polyarthritis. Mucocutaneous involvement, manifested
by painless oral ulcers, balanitis, and keratoderma
blennorrhagica, is frequently present. The etiology of
Reiter’s syndrome is uncertain, but it is commonly
considered to be a “reactive” phenomenon to enteric
or venereal infections in genetically susceptible individuals. Conventional features of autoimmune phenomena have not been described in patients with this
illness. We report the occurrence of pericarditis and
cardiac tamponade in a patient with Reiter’s syndrome
whose subsequent course and laboratory investigations showed features more commonly encountered in
systemic lupus erythematosus.
Case report. A 29-year-old white man was admitted to the Manhattan Veterans Administration
Hospital in September 1980. In 1972 the patient had
developed urethritis and was treated with penicillin for
presumed gonorrhea. Several weeks after this episode
he was hospitalized with bilateral ankle arthritis and
heel pain. During the subsequent 8 years, he suffered
from intermittent conjunctivitis, low back pain, heel
pain, and arthritis of the wrists, fingers, and toes.
These symptoms were partially controlled with aspirin
and indomethacin. Sacroiliitis was documented radiographically (Figure 1). The diagnosis of Reiter‘s synFrom the Division of Rheumatology. New York University
Medical Center.
Paul S. Aisen, MD: Fellow; Bruce N . Cronstein. MD:
Instructor; Sara B. Kramer, MD: Fellow.
Address reprint requests to Paul S. Aisen. MD. Division of
Rheumatology. Department of Medicine, New York University
Medical Center, SSO First Avenue, New York. NY 10016.
Submitted for publication January 24. 1983: accepted in
revised form July 22. 1983.
Arthritis and Rheumatism, Vol. 26, No. I1 (November 1983)
drome was made in 1975. Complete histocompatibility
antigen determination revealed A1 1, Aw24, B13, B27,
Cwl, Cw2, and DRw6.
Three weeks prior to admission, the patient
developed pleuritic chest pain and dyspnea on exertion. These symptoms progressed until he sought
emergency medical attention. He had been taking
maintenance doses of indomethacin and enteric-coated aspirin. There was no family history of arthritis or
collagen-vascular disease.
On physical examination. his temperature was
102”F, pulse 130, respirations 40, and blood pressure
110/70 with 30 mm pulsus paradoxicus. Neck veins
were distended. The lungs were clear, heart sounds
were distant, and no murmurs or rubs were audible.
The liver span was 16 cm. The wrists, metacarpophalangeal, and proximal interphalangeal joints were
tender without frank arthritis. No skin rash, conjunctivitis, oral or genital lesions were noted.
Initial laboratory data included: hematocrit
3696, white blood cell count 15,000/mm3(65% neutrophils, 995 bands, 25% lymphocytes, 1% monocytes),
platelet count 65,000/mm3, urinalysis results normal,
erythrocyte sedimentation rate 100 mm/hour. prothrombin time 18.0 seconds with a control of 11.9
seconds, and partial thromboplastin time 71.8 seconds
with a control of 34.8 seconds.
Electrocardiogram revealed a sinus tachycardia
with low voltage. Chest roentgenogram demonstrated
a grossly enlarged, water bottle-shaped heart (Figure
2). An echocardiogram confirmed the presence of a
large pericardial effusion and showed paradoxical motion of the interventricular septum, suggestive of cardiac tamponade. A pericardial window was placed,
and 1,300 cc of sterile, bloody exudative fluid was
Figure 1. Radiographs of sacroiliac joints, demonstrating sacroiliitis. 1975. A , Right. B , Left.
drained. The biopsy specimen showed nonspecific
fibrinous pericarditis.
Subsequent laboratory findings included: positive antinuclear antibodies (ANA) at 1 : 160 (speckled
pattern), positive antibody to extractable nuclear antigen at 1 : 128, negative anti-Sm antigen, negative antiPNA antibodies, negative latex fixation, negative lupus erythematosus test, C3 74 mg/dl (normal 86-244).
C4 9 mg/dl (normal 15-66), negative VDRL, negative
PPD, and no increase in viral antibody titers. Mixture
of the patient’s plasma with an equal volume of normal
plasma yielded a prothrombin time of 13.9 seconds
with a control of 12.3 seconds, and a partial thromboplastin time of 69.5 seconds with a control of 42.4
seconds, indicating a circulating anticoagulant. Direct
Coombs’ test was positive for IgG. Bone marrow
analysis revealed increased numbers of megakaryocytes and erythroid precursors. Immunofluorescence
examination of both sun-exposed and non-sun-exposed skin revealed granular deposits of IgG and IgM
at the dermal-epidermal junction.
After removal of the drainage tube, the pericardial effusion reaccumulated and an exudative right
pleural effusion developed. The patient was placed on
a regimen of prednisone 60 mg daily; this led to
symptomatic improvement, decrease in effusions, and
increase in platelet count. He was discharged on the
seventeenth hospital day.
Since discharge, the prednisone dosage has
been tapered to as low as 5 mg daily. The patient has
had several exacerbations of disease activity consisting of pleuritis with pleural effusions, myalgias, and
arthralgias. He has had 1 episode of painless oral
ulcers; conjunctivitis and urethritis have not recurred.
Clinical disease activity has been accompanied by
hypocomplementemia, and both have responded to
increased oral steroid therapy.
Discussion. The diagnosis of Reiter’s syndrome
in this patient was made 5 years prior to the current
admission, on the basis of the classic triad of features,
plus sacroiliitis. HLA-B27 was present in this patient.
as in approximately 80% of whites with Reiter’s syndrome (1). Eight years after the onset of his illness, our
patient developed severe pleuropericarditis requiring
pericardial drainage, anemia with a positive Coombs’
test, thrombocytopenia, positive ANA, positive lupus
band test, and hypocomplementemia.
Cardiac manifestations, the most common of
Figure 2. Portable radiograph of the chest, demonstrating the enlarged water bottle-shaped
cardiac shadow, 1980.
which are conduction disturbances and aortic insufficiency, occur in less than 10% of patients with Reiter’s
syndrome (2-8). Symptomatic pericarditis and, more
rarely, pleuritis have been reported, usually during the
acute phase of the illness (2,9). Cardiac tamponade has
not been described in Reiter’s syndrome. In our patient, pleuropericarditis was a late occurrence and was
accompanied by multiple hematologic and serologic
abnormalities indicative of an autoimmune disorder.
Positive lupus band tests on biopsies of clinically normal skin are reported in 36-100‘% of patients
with systemic lupus erythematosus (10). If bullous
pemphigoid is excluded, this test has been uniformly
negative in other illnesses, though few patients with
spondylarthropathies have been studied ( I 1.12). Previous studies of Reiter’s syndrome have demonstrated
normal serum complement levels (13,14) and the absence of antinuclear antibodies (7). On the basis of the
clinical and laboratory findings in this patient, we
attributed the polyserositis not to Reiter’s syndrome
but to systemic lupus erythematosus. The patient
meets the revised American Rheumatism Association
criteria for inclusion in clinical studies of systemic
lupus erythematosus, with the presence of arthritis,
serositis, oral ulcers, thrombocytopenia, and an abnormal titer of antinuclear antibody during the course
of his illness (IS).
Overlap syndromes among the rheumatic illnesses have been widely reported. These include
mixed connective tissue disease with features of
scleroderma, lupus, and polymyositis, and coexisting
rheumatoid arthritis and lupus. Recent reports cite the
progression of juvenile arthritis to systemic lupus
(16.17). A patient with definite ankylosing spondylitis
who developed lupus has been described (18), as well
as others with the combination of sacroiliitis, HLAB27, and positive ANA (19). One study reports that
antinuclear factors can be demonstrated in 70% of
patients with ankylosing spondylitis when human leukocytes are used as substrate (while conventional
ANA tests were negative in all patients studied),
suggesting that autoimmunity may play a role in that
illness (20). The relationship between the spondylarthropathies and systemic lupus erythematosus remains
This is the first report of lupus and Reiter‘s
syndrome occurring in the same patient. Features
common to both Reiter’s and lupus include polyarthritis, stomatitis, pericarditis, and diverse peripheral and
central nervous system manifestations. It is therefore
conceivable that lupus complicating Reiter’s may have
been overlooked in other instances.
Acknowledgments. The authors wish to thank Dr.
Robert Winchester for histocompatibility antigen determinations, and Drs. Joel Buxbaum and Gerald Weissmann for
reviewing the manuscript.
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lupus, patients, reiter, syndrome, systemic, erythematosus
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