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Sickle cell crisis following intraarticular steroid therapy for rheumatoid arthritis.

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We describe 2 patients who had coexistent rheumatoid arthritis and sickle cell disease. Both patients
developed sickle cell crises following intraarticular injection of corticosteroids to control their arthritis. The
mechanism of this phenomenon is not clear, but it is
suggested that intraarticular steroids be used with caution in patients with sickle cell disease.
Sickle cell disease and sickle cell trait are both
associated with articular manifestations. Gout and
hyperuricemia, aseptic necrosis, hemarthrosis, and
more recently, an inflammatory arthropathy have been
reported to occur in patients (1-3). The latter is
thought to occur during sickle cell crisis and usually
responds to treatment of the crisis (2). We have
recently encountered 2 patients with coexisting sickle
cell disease and rheumatoid arthritis (RA), in whom
sickle cell crisis appears to have been precipitated by
the intraarticular administration of steroids.
Patient 1. Patient 1, a 53-year-old Jamaican
woman with known sickle cell trait, first presented
with right-sided chest pain and pain in the right arm in
October 1980. She was treated with transfusions. She
From the Rheumatic Disease Unit, University of Toronto,
The Wellesley Hospital, and Women’s College Hospital, Toronto,
Ontario, Canada.
Dafna D. Gladman, MD, FRCP(C): Associate Professor of
Medicine, University of Toronto, Rheumatic Disease Unit, The
Wellesley Hospital, and Women’s College Hospital; Claire Bombardier MD, FRCP(C): Associate Professor of Medicine, University of
Toronto and The Wellesley Hospital.
Address reprint requests to Dr. D. Gladman, Women‘s
College Hospital, Burton Hall, 60 Grosvenor St., Suite 423,
Toronto, Ontario M5S 1B6, Canada.
Submitted for publication October 29, 1985; accepted in
revised form January 15, 1987.
Arthritis and Rheumatism, Vol. 30, No. 9 (September 1987)
was referred for rheumatologic consultation in November 1980, because of polyarthritis that involved
hands, wrists, feet, shoulders, and knees, and morning
stiffness of 2 hours duration.
Physical examination revealed 27 actively inflamed joints, 13 effusions, and a reduced grip strength
of 90 mm Hg. Laboratory tests revealed a hemoglobin
level of 9.7 gm/dl, white blood cell count of 12.9 x
109/liter, platelet count of 780 x IO’Aiter, and an
erythrocyte sedimentation rate of 124 m d h o u r . There
was marked eosinophilia of 3 ,090/mm3. Hemoglobin
electrophoresis showed 68% hemoglobin S and 32%
hemoglobin A. Latex fixation test for rheumatoid
factor (RF) was positive (titer 1:5,120). The gamma
globulin level was elevated at 2.2 gm/dl. Investigation
of eosinophilia failed to reveal any parasites. Radiographs of the hands showed soft tissue swelling, as
well as erosive changes compatible with RA (Figure
1). No sickle cell changes were detected in the bones.
She was treated with enteric-coated acetylsalicylic
acid (ECASA) and an intraarticular injection of
methylprednisolone (Depo-Medrol) into a shoulder
joint and an ankle joint. Gold therapy was complicated
by proteinuria and was discontinued after a month.
She was started on a regimen of prednisone, 7.5 mg/day.
On February 3, 1981, she presented with a large
knee effusion and a ruptured Baker’s cyst. After the
fluid was aspirated, the right knee was injected with 80
mg of Depo-Medrol. Twenty-four hours later, she
presented to the emergency room with severe back
pain and a hemoglobin level of 7.7 g d d l , which later
dropped to 4.3 gm/dl. She responded to treatment with
oxygen, hydration, and 3 transfusions of packed red
blood cells.
She remained well until July 4, 1981, when she
presented with a rheumatoid nodule on her right elbow
Figure 1. Radiograph of the hands of patient 1 , showing soft tissue swelling over several proximal
interphalangeal joints and erosions of the ulnar styloid on the left hand, as well as an early erosion of the
second right metacarpophalangeal joint.
and a recurrent effusion in the right wrist. Within 12
hours of intraarticular injection, she again presented to
the emergency room with abdominal pain, pleuritic
chest pain, and back pain. These symptoms responded
to hydration, oxygen therapy, and analgesic treatment. Therapy with chloroquine was begun for RA.
Between August 1981 and January 1982 she continued
to have mild arthritis and was maintained on a therapeutic regimen of nonsteroidal antiinflammatory
drugs, chloroquine, and low-dose prednisone.
In April 1982, she had another sickle cell crisis,
and avascular necrosis of the right hip was detected.
She underwent a total right hip replacement in September 1982. There have been no further crises since
then. She now has persistent pain in her right ankle,
but she is not taking chloroquine or prednisone.
Patient 2. The patient, a 30-year-old Guyanese
woman with a 6-year history of sickle cell disease
(hemoglobins S and D), first presented with arthritis of
the right knee in February 1979. The latex fixation test
for RF was positive (1 :320). The knee was aspirated,
and 40 mg of Depo-Medrol was injected. Two days
later, she presented with bone pain in her extremities
and her back, a hemoglobin level of 9 gm/dl, and
leukocytosis of 27.3 x lO’/liter. Hemoglobin electrophoresis revealed 95% hemoglobin S. She was admitted to the hospital and treated for sickle cell crisis.
Over the ensuing few months, she continued to have
persistent arthritis in the knee, and she developed
progressive small and large joint polyarthritis.
Physical examination revealed 10 actively inflamed joints and 4 effusions. The RF was then positive at a titer of 1:1,280. Radiographs of the hands
showed changes typical of RA (Figure 2). Her right
knee was again aspirated. The fluid showed a white
blood cell count of 34.8 x 109/literand an RF titer of
1:640. Bacterial and fungal cultures were negative.
Steroid was again injected intraarticularly . Twelve
hours later, the patient presented to the emergency
room with a sickle cell crisis. The RA was only
marginally responsive to ECASA therapy, and she
was admitted for further treatment.
While in the hospital in January 1980, with 11
actively inflamed joints and 8 effusions, she had an
additional intraarticular steroid injection. Again,
within 2 days she experienced severe back pain,
anemia, and marked leukocytosis. She was treated
with oxygen, analgesics, and blood transfusions, and
Figure 2. Radiographof the hands of patient 2, showing typical erosive changes of rheumatoid arthritis in the
wrists, with resultant radial deviation of the wrist. Erosive changes and ulnar deviation of the metacarpophalangeal joints are also seen.
she was started on a regimen of sodium aurothiomalate
Over the next few months her RA showed
marked improvement. She became pregnant; delivery
was by cesarean section. This was complicated by an
infection and another crisis. However, since October
1981, she has not required further admission to the
These 2 cases illustrate the occurrence of RA in
patients with sickle cell disease. We have not found
any previous reports of the coexistence of RA with
sickle cell disease, although Schumacher et a1 (4)
described 2 patients with erosive destructive joint
disease whose synovial pathologic findings were similar to those in RA. Earlier reports of inflammatory
arthritis in patients with this disease suggested that the
polyarthritis may have been a manifestation of the
sickle cell crisis. Our patients fulfilled the American
Rheumatism Association revised criteria for definite
RA (5) (polyarthritis, positive rheumatoid factor, rheumatoid nodules, and erosive changes).
These cases also demonstrate the apparent pre-
cipitation of a crisis by the intraarticular injection of
steroids. In the first patient, the first 2 steroid injections were uneventful, as was oral steroid administration. However, the last 2 injections were followed by
the development of a crisis within 12-24 hours of
injection. In this case, it is possible that the worsening
of arthritis represented an early manifestation of sickle
cell crisis, although the crisis did not become evident
until after steroids were injected intraarticularly.
In the second patient, 3 intraarticular injections
of Depo-Medrol were followed by sickle cell crisis
within 24-48 hours of injection. This patient did not
receive an intraarticular injection without the subsequent development of a sickle cell crisis. Although it
might be possible that active arthritis precipitated the
crisis, the crisis did not occur until after the intraarticular injection of corticosteroid.
There is no evidence that oral steroid therapy
causes sickle cell crises in patients who have sickle
cell disease without RA. Furthermore, in our 2 patients, the crisis occurred after intraarticular steroid
injection for control of the arthritis. The frequency of
the crises seemed to decrease when steroid injections
were no longer given.
The mechanism of this apparent precipitation of
a crisis by intraarticular steroid therapy is unclear. It is
unlikely that steroids given intraarticularly would alter
the blood pH, oxygen saturation, or any other known
mechanism that precipitates the sickling process. It is
suggested, however, that intraarticular steroids be
given with caution in patients with sickle cell disease.
I . Schumacher HR, Andrews R, McLaughlin G: Arthropathy in sickle cell disease. Ann Intern Med 78:203-211,
2. Espinoza LR, Spilberg I, Osterland CK: Joint manifestations of sickle cell disease. Medicine (Baltimore) 53:
295-305, 1974
3. Schumacher HR: Arthritis associated with sickle cell
disease and other hemoglobinopathies, Textbook of
Rheumatology. Edited by WN Kelley, ED Harris Jr, S
Ruddy, CB Sledge. Philadelphia, WB Saunders, 1981, pp
4. Schumacher HR, Donvart BB, Bond J, Alavi A, Miller
W: Chronic synovitis with early cartilage destruction in
sickle cell disease. Ann Rheum Dis 36:413-419, 1977
5. Ropes MW, Bennett GA, Cobb S, Jacox R, Jessar RA:
1958 revision of diagnostic criteria for rheumatoid arthritis. Bull Rheum Dis 9:175-176, 1958
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sickle, steroid, following, arthritis, crisis, therapy, rheumatoid, cells, intraarticular
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