RARE PRESENTATION OF LUPUS: SLE PRESENTING AS SEPTIC SHOCK
Henry Ford Health System
Introduction: Infections are common in patients with SLE because of the immune impairments, but there are only very few cases that present as infection as the initial manifestation of SLE. Here we pre..more »
Introduction: Infections are common in patients with SLE because of the immune impairments, but there are only very few cases that present as infection as the initial manifestation of SLE. Here we present the case of a patient whose initial presentation of SLE was septic shock secondary to Pseudomonas UTI complicated by Pseudomonas bacteremia.
Case presentation: The patient is an 18-year-old female with no significant past medical history presented with fever, chills, nausea, episodes of vomiting and bilateral lower extremity edema. Vital signs showed that she is tachycardic to 130s, tachypneic to 30s. Physical exam showed tense bullae and purpuric plaques bilaterally in lower extremities. Labs were significant for lactic acid of 4.2 and creatinine of 4.54, hemoglobin of 7.1. Infectious workup was done due to concern of septic shock and the patient was found to have UA showing more than 182 WBCs, 114 RBCs, and more than 500 proteinuria. Blood cultures were positive for gram-negative bacilli. The patient was started on broad-spectrum antibiotics and was admitted to MICU for septic shock. Patient was intubated and started on Levophed.
Further workup included: Urine culture growing Pseudomonas. Blood cultures growing pan-susceptible Pseudomonas. BMP showing up rising creatinine. Autoimmune workup: positive ANA(1:1280) homogeneous pattern, double-stranded DNA antibodies(1:320), SS-A, SS-B, ENA smith/RNP low complement levels, negative C ANCA, p-ANCA, CCP, and anti-GBM antibodies. Echocardiogram showed moderately sized pericardial effusion is present circumferentially around the entire heart. Skin biopsy of tense bullae showed thrombotic vasculopathy with widespread dermal necrosis- most likely represents septic vasculopathy. Skin biopsy tissue culture was positive for Pseudomonas. Renal biopsy showed Diffuse Lupus Nephritis, Class 4-G (A).
Management: The patient was treated with dialysis (initially SLED and later hemodialysis). She also received Cefepime for Pseudomonas infection. She got Solumedrol 500 mg daily for 3 days and that was transitioned to 60mg daily. She was also started on Cellcept given the superiority and better safety compared to Cyclophosphamide for induction therapy in the African-American population, especially with childbearing age.
Discussion: SLE is a chronic inflammatory multisystem disease with immunological abnormalities and seen more often in women than men. In the literature, the infection has been reported as the leading cause of death in a patient with SLE but there are very few cases of SLE which present as infection as the initial manifestation. There was an almost 5-fold increase in the risk of death from infection in patients with SLE when compared with the general population. Factors contributing to this include SLE causing neutropenia, lymphopenia, hypocomplementemia, renal involvement, neuropsychiatric manifestations, and the use of glucocorticoids and other immunosuppressive drugs.
Conclusion: Physicians should always consider the possibility of complement deficiencies in young adult patients presenting with severe infections and the presence of hypocomplementemia should suggest the possibility of SLE, even in patients with no prior history of SLE and patient should undergo further workup to diagnose Lupus. In this situation, the patient should be treated for both infection and SLE.
Henry Ford Hospital
Resident PGY 2
Henry Ford Health System