RESUMEN
Bullous pemphigoid (BP) is a rare autoimmune blistering disease caused by pathogenic antibodies which deposit into the dermo-epidermal junction. We present a case of bullous pemphigoid in a young female refractory to initial therapy with immunosuppressives and steroids who experienced significant improvement in blister burden after a course of adjunct therapeutic plasma exchange (TPE). This case report provides valuable insight into the benefit of utilizing TPE for BP and provides treatment specifics regarding use of TPE in a patient with BP.
Asunto(s)
Enfermedades Autoinmunes , Penfigoide Ampolloso , Femenino , Humanos , Penfigoide Ampolloso/terapia , Intercambio Plasmático/efectos adversos , Vesícula/patología , Vesícula/terapia , Enfermedades Autoinmunes/terapia , Inmunosupresores/uso terapéuticoRESUMEN
Acute kidney injury (AKI) has been seen in patients hospitalized with a SARS-CoV-2 (COVID-19) infection,but the pathophysiology of glomerular injury is not yet fully understood. We present a case of COVID-19-related "glomerular endotheliosis" in which a 51-year-old female with a 13-year history of class IV lupus nephritis was admitted for COVID-19 pneumonia. Her lupus nephritis had been in complete renal remission for the past 10 years with a baseline serum creatinine level of 1.3 mg/dL and no proteinuria. Her serological workup, including complement levels, was unremarkable. Due to the worsening renal function and persistent proteinuria, she underwent a kidney biopsy that revealed diffuse glomerular endothelial cell swelling, also known as glomerular endotheliosis. Her clinical course unfortunately deteriorated and she succumbed to acute respiratory distress syndrome. As circulating anti-angiogenic factors may contribute to the pathogenesis of endothelial dysfunction leading to glomerular endotheliosis, we propose that a similar circulating antiangiogenic factor may have been triggered by COVID-19 and played a role in our patient's progressive renal failure.