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.