14 April 2020 : Case report
Lupus-Induced Vasculitis and Multiple Organ Dysfunction Syndrome as the First Presentation of Systemic Lupus Erythematosus (SLE) in Pregnancy
Rare diseasePatrick A. Yousif1ABCDEF*, Hameadreza Moshrefi2ABCDEF, Alireza Meysami2CDG, Ayad H. Alkhatib2DG
Am J Case Rep 2020; 21:e921299
BACKGROUND: Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by autoantibody production leading to inflammation in multiple organs; it commonly affects young women in their child-bearing years. Clinical manifestations are diverse and range from mild arthritis to diffuse alveolar hemorrhage (DAH). DAH is a rare and devastating complication of SLE that carries a mortality rate of up to 50%, despite aggressive therapy.
CASE REPORT: A 21-year-old primigravida at 16 weeks gestation presents with a productive cough, rash, sore throat, and high-grade fever. Chest x-ray suggested multifocal pneumonia. Patient deteriorated despite antibiotics and intravenous (IV) fluids. She developed worsening anemia, leukopenia, and thrombocytopenia. Autoimmune workup was positive for Coombs, antinuclear antibody, anti-smith antibody, and hypocomplementemia. Skin biopsy was consistent with SLE. SLE vasculitis was suspected. She required mechanical intubation for rapid respiratory deterioration, with CT thorax suggesting ARDS. Bronchoscopy was done and confirmed DAH. Her course was further complicated with retinopathy and acute pancreatitis associated with SLE. She was treated with IV steroids, IV cyclophosphamide, and plasmapheresis, with significant clinical improvement and successful extubation. She delivered a healthy baby at 32 weeks gestation.
CONCLUSIONS: Early recognition and initiation of treatment is critical to survival in DAH and requires a high index of clinical suspicion. Treatment includes high-dose steroids, cyclophosphamide, and plasma exchange. Pregnancy increases the risk of adverse outcome in SLE. Seven cases of DAH in pregnant patients with SLE have been reported. Here, we report a catastrophic presentation of DAH, acute pancreatitis, and retinopathy in a pregnant patient with newly diagnosed SLE.
Keywords: Hemorrhage, Lupus Erythematosus, Systemic, Pregnancy, Lung Diseases, Multiple Organ Failure, Pregnancy Complications, Pulmonary Alveoli, Vasculitis, young adult
27 Jan 2023 : Case reportA 37-Year-Old Man with Familial Hereditary Hemorrhagic Telangiectasia and Pulmonary Arteriovenous Malformat...
Am J Case Rep In Press; DOI: 10.12659/AJCR.938548
26 Jan 2023 : Case reportA 39-Year-Old Woman with Endometriosis Who Developed a Subcapsular Liver Hematoma Following Laparotomic Sur...
Am J Case Rep In Press; DOI: 10.12659/AJCR.938169
26 Jan 2023 : Case reportOptic Neuritis as a Consequence of Long-Term Medical Treatment Using Anastrozole: A Case Report
Am J Case Rep In Press; DOI: 10.12659/AJCR.939026
Most Viewed Current Articles
13 Jul 2022 : Case reportWhistling Scrotum: An Unusual Presentation of Pneumomediastinum in the Setting of an Open Scrotal Wound
Am J Case Rep 2022; 23:e936441
06 Dec 2021 : Case reportLipedema Can Be Treated Non-Surgically: A Report of 5 Cases
Am J Case Rep 2021; 22:e934406
23 Feb 2022 : Case reportPenile Necrosis Associated with Local Intravenous Injection of Cocaine
Am J Case Rep 2022; 23:e935250