Case Presentation
A 38-year-old woman complaining of bloody diarrhea and general fatigue
was admitted to the emergency department and was then hospitalized. Her
previous medical record included upper gastrointestinal endoscopy and
colonoscopy that revealed crypt destructive colitis and chronic
gastritis. With these findings, she was diagnosed with early UC and was
under treatment with mesalazine (3g daily) and pantoprazole (40mg
daily). In addition, her routine blood tests had revealed pancytopenia
before. This had led the doctor to obtain a bone marrow biopsy which was
inconclusive due to an unsatisfactory sample, and also to request
rheumatologic laboratory tests which were reported negative.
The patient showed symptoms of general fatigue and lethargy along with
fever and night sweats. She also stated that she had involuntarily lost
14 kg in 3 months. Her family members also stated that their younger
sibling also had a history of an unknown rheumatologic disease and was
expired years ago. Her physical examinations showed bilateral pitting
edema in both legs, oral ulcers. she also suffered from bloody diarrhea
and hemoptysis. When she was admitted she was severely ill. Her lower
limb forces were reduced (3/5) and she could barely stand or walk. Her
neck examination showed enlarged lymph nodes on the back of her neck.
However, her vital signs were stable. As the patient’s clinical signs
were suggestive for SLE or myositis we ordered prednisolone (1 mg/kg)
before the laboratory results were ready. This significantly improved
limb forces and hemoptysis.
Her laboratory tests were as shown in the table (Table 1, 2). The
significant findings were pancytopenia, mildly elevated liver enzymes, a
high ESR, severely low reticulocyte count, high LDH and B12 levels. Her
urine analysis revealed hematuria and bacteriuria. Her arterial blood
gases were normal. As her hemoglobin levels were deemed too low, she
received 1 unit of packed RBC. We also obtained another bone marrow
biopsy which was not in favor of malignancies. Another notable and
important finding is that her serum protein electrophoresis revealed an
unusual abnormality which was increased gamma globulin levels while
albumin levels were low (Table 3). This presentation was suggestive of
gammopathy.
In her following work up a spiral chest computed tomography (CT) scan
without contrast was requested that revealed ground-glass opacities in
the lower lobes of both lungs. This finding was more prominent in the
left lung. This finding was also suggestive of pneumonia. Other
remarkable findings were mosaic attenuation in the parenchyma of both
lungs, air cyst (12 mm ×12 mm) in
left upper lobe, bilateral mild pleural effusion, cardiomegaly, and mild
pericardial effusion.
As the findings were compatible with pneumonia, antibiotic therapy was
initiated and she received 4.5 gr of piperacillin/tazobactam
intravenously 3 times a day and 750 mgr of levofloxacin daily orally.
Due to swelling and edema in her lower limbs, she underwent venous color
doppler sonography and nothing remarkable was observed. the patient also
underwent neck sonography and many reactive lymph nodes were found on
both sides of the neck. the largest ones were (30 mm ×9.5 mm) in zone 2
of the right side and the other (23 mm × 7 mm) in zone 2 of the left
side. Other sonographic evaluations revealed axillary lymph nodes as big
as (31mm ×15 mm) on the left and (25mm ×17 mm) on the right
respectively.
She also underwent echocardiography to assess cardiac activity and the
prominent finding was a decreased ejection fraction (EF) (45%). As her
medical condition and the hematochezia had worsened 5mg of prednisolone
3 times a day and also a single unit of IVIG 60mg was ordered.
As the clinical manifestations of the patient were consistent with SLE,
a rheumatologic consultation was requested. Which suggested laboratory
tests that turned out to be positive for anti-dsDNA and also her urine
analysis manifested severe proteinuria. Meanwhile, she had already shown
symptoms of polyserositis, mouth ulcers, and pancytopenia. With all the
aforementioned findings and signs, SLE became a confirmed diagnosis for
the patient. Thus, intensive therapy with rituximab induction therapy
along with 500mg of prednisolone pulse therapy for 3 days was started,
we also used 200 mg of hydroxychloroquine daily simultaneously with a
daily dose of 1.5 gr of mycophenolic acid as a maintenance therapy.
Unfortunately, though, 2 days after the initiation of therapy oxygen
saturation levels suddenly dropped to 70% and the patient became
dyspneic thus the patient was intubated. Unfortunately, the patient
showed signs of hemoptysis which was suggestive of alveolar hemorrhage.
She was intubated and 2 units of fresh frozen plasma (FFP) were
transfused. An emergent chest X-RAY was obtained that showed diffused
patchy infiltrates that resembled either pneumocystis pneumonia (PCP),
alveolar hemorrhage, or pulmonary thromboembolism (PTE). Portable
echocardiography showed an even more decreased EF (40%) along with mild
right ventricle enlargement and moderate left ventricle dysfunction.
thereafter a new course of antibiotic therapy started with intravenous
(IV) cotrimoxazole, vancomycin, and cefepime. The patient received
another 500 mg of methylprednisolone pulse therapy and 4 mg of
dexamethasone IV, 3 times a day. However, the patient expired on the
same day due to alveolar hemorrhage arrest.