April Nelsen, MS, MLS(ASCP)CM; Rosemarie Clauson, MLS(ASCP)CM; Kassi Erickson, MS, MLS(ASCP)CM; Stacie Lansink, MS, MT(ASCP); and Tiffany Niemann, MLS(ASCP)CM
After weeks of shortness of breath and sharp chest pain, a 48-year-old Caucasian female presented to the ED in a hypertensive state; her blood pressure was 193/113. Her history revealed preeclampsia in a previous pregnancy resulting in acute renal failure and multiple pulmonary emboli. A post-partum renal biopsy showed thrombotic microangiopathy, in which the patient underwent dialysis and anticoagulation therapy. Five years post-partum, the patient was diagnosed with Systemic Lupus Erythematosus (SLE). At the time of SLE diagnosis, the patient tested negative for dsDNA and Sm/RNP antibodies but presented with a positive Antinuclear Antibodies (ANA) and antiphospholipid antibodies.
During the patient’s ED admission, a S-T elevation on an electrocardiogram (EKG) and diffuse ground glass bilateral pleural effusions on the computerized topography (CT) scan were observed. Metoprolol, heparin, and aspirin were immediately administered. Admission labs revealed progressive renal failure, anemia, thrombocytopenia, and prolonged coagulation results. Arterial Blood Gases showed the patient in a state of metabolic acidosis. One unit of packed red blood cells was transfused due to the patient’s anemia. A bronchoalveolar lavage (BAL) produced a red, turbid fluid. Cultures and respiratory pathogen serological tests came back negative. The bronchoscopy displayed bloody effusions in both the right and left upper lobes, suggestive of a diffuse alveolar hemorrhage. An EKG noted a mitral valve vegetation, known as Libman-Sacks endocarditis in SLE patients, and was also causing mitral valve regurgitation.
Due to the concern for advancing the alveolar hemorrhage, anti-coagulant therapy was delayed. To determine if the prolonged coagulation was due to a lupus anticoagulant or not, coagulation tests were sent to Mayo, but came back inconclusive. Tests for anticardiolipin and anti-beta 2 glycoprotein I antibodies (IgM and IgG isotypes) were recommended and came back weakly positive but were diagnostic of a catastrophic presentation of Antiphospholipid Syndrome (APS).
This diagnosis was consistent with the patient’s history of preeclampsia, seizures, mitral valve regurgitation (exacerbated by the Libman-Sacks endocarditis caused by SLE), hypertension, and increased protein in the urine. The patient’s kidneys continued to deteriorate rapidly, and the alveolar hemorrhage soon became a complication, so the patient was transferred to the transitional ICU and pulse dose steroid, along with oxygen therapy, were initiated.
The patient’s renal function soon digressed to Stage 4 Chronic Kidney Disease (CKD) and she was transferred to a hospital more equipped to address her complications. The diagnosis of SLE, despite being negative for all common autoantibodies associated with SLE, was unusual. The added catastrophic APS is a rare variant of the disease, and it resulted in the failure of multiple organs. The prognosis for this patient is poor with a renal transplant being the only long-term solution.
April Nelson is an instructor. Kassi Erickson is an instructor. Stacie Lansink is program director. Tiffany Niemann is laboratory support specialist. They are all with the Medical Laboratory Science Program at South Dakota State University in Brookings, South Dakota.
Rosemarie Clauson is an MLS-generalist at Jamestown Regional Medical Center in Jamestown, North Dakota.