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"I'm Sick" How Sick? A Rare Presentation of Autoimmune Crisis
Description
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A3410 - ""I'm Sick"" How Sick? A Rare Presentation of Autoimmune Crisis
Author Block: C. Dahn, H. Gandhi, T. T. Bellamkonda; Albert Einstein College of Medicine, Montefiore Medical Center, Bronx, NY, United States.
Introduction:
A wide array of vague symptoms, various organ system involvement, and a complex illness severity spectrum encompass autoimmune emergencies. This is complicated by potential superimposed processes like sepsis and thrombotic microangiopathy (TMA) such as thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). Autoimmune crises are rare with systemic lupus erythematosus (SLE) being most common; however, they can generate significant mortality upwards of 55% in autoimmune crisis and up to 90% in untreated TMA.
Case Presentation:
A 25 year-old woman with intellectual disability, seizures, depression, and asthma presented to the Emergency Department complaining of general malaise and myalgia. Her initial exam was notable for tachycardia, extensive oral ulcers, and facial erythema with purpuric lesions. Her initial labs showed pancytopenia, hyponatremia, elevated liver enzymes, and an elevated creatinine kinase. Over the next few days diagnostic workup revealed positive dsDNA antibody, decreased complement levels C3 and C4, elevated ANA, positive Smith antibody, positive SSA/Ro antibody, and a max CK of 26,900. She was treated with prednisone 1 mg/kg/day for autoimmune disease of uncertain type and admitted to the ICU on hospital day seven for closer monitoring of electrolytes where she rapidly progressed overnight to acute hypoxic and hypercapnic respiratory failure (pH of 6.8) requiring intubation, acute respiratory distress syndrome (ARDS), profound shock, acute kidney injury requiring continuous veno-venous hemofiltration, and worsening pancytopenia. She was treated with high dose pulse steroids, broad-spectrum antimicrobials for sepsis, epoprostenol in case of pulmonary hypertension/vasculopathy, as well as plasmapheresis for SLE with overlap syndrome such as myositis and/or an undifferentiated TMA while confirmatory tests were pending. Skin biopsy and ADAMTS13 level > 20 % were consistent with HUS or atypical HUS. Hemodynamics and gas exchange improved within 24 hours, but pancytopenia and AKI persisted. Consideration of eculizumab was discussed but patient improved prior to initiation. The patient was eventually discharged to home after a long hospital stay.
Discussion:
This case illustrates a complicated clinical presentation of SLE and HUS. As rare entities with delayed confirmatory testing, early clinical recognition is critical in order to maximize supportive care. Steroids are first line treatment for SLE, with other immunosuppressants and plasmapheresis as options, but no Level I evidence exists to guide therapy. Current guidelines recommend plasmapheresis initiation within 4 to 8 hours when TMA is suspected and eculizumab is considered standard care for HUS.
Author Block: C. Dahn, H. Gandhi, T. T. Bellamkonda; Albert Einstein College of Medicine, Montefiore Medical Center, Bronx, NY, United States.
Introduction:
A wide array of vague symptoms, various organ system involvement, and a complex illness severity spectrum encompass autoimmune emergencies. This is complicated by potential superimposed processes like sepsis and thrombotic microangiopathy (TMA) such as thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). Autoimmune crises are rare with systemic lupus erythematosus (SLE) being most common; however, they can generate significant mortality upwards of 55% in autoimmune crisis and up to 90% in untreated TMA.
Case Presentation:
A 25 year-old woman with intellectual disability, seizures, depression, and asthma presented to the Emergency Department complaining of general malaise and myalgia. Her initial exam was notable for tachycardia, extensive oral ulcers, and facial erythema with purpuric lesions. Her initial labs showed pancytopenia, hyponatremia, elevated liver enzymes, and an elevated creatinine kinase. Over the next few days diagnostic workup revealed positive dsDNA antibody, decreased complement levels C3 and C4, elevated ANA, positive Smith antibody, positive SSA/Ro antibody, and a max CK of 26,900. She was treated with prednisone 1 mg/kg/day for autoimmune disease of uncertain type and admitted to the ICU on hospital day seven for closer monitoring of electrolytes where she rapidly progressed overnight to acute hypoxic and hypercapnic respiratory failure (pH of 6.8) requiring intubation, acute respiratory distress syndrome (ARDS), profound shock, acute kidney injury requiring continuous veno-venous hemofiltration, and worsening pancytopenia. She was treated with high dose pulse steroids, broad-spectrum antimicrobials for sepsis, epoprostenol in case of pulmonary hypertension/vasculopathy, as well as plasmapheresis for SLE with overlap syndrome such as myositis and/or an undifferentiated TMA while confirmatory tests were pending. Skin biopsy and ADAMTS13 level > 20 % were consistent with HUS or atypical HUS. Hemodynamics and gas exchange improved within 24 hours, but pancytopenia and AKI persisted. Consideration of eculizumab was discussed but patient improved prior to initiation. The patient was eventually discharged to home after a long hospital stay.
Discussion:
This case illustrates a complicated clinical presentation of SLE and HUS. As rare entities with delayed confirmatory testing, early clinical recognition is critical in order to maximize supportive care. Steroids are first line treatment for SLE, with other immunosuppressants and plasmapheresis as options, but no Level I evidence exists to guide therapy. Current guidelines recommend plasmapheresis initiation within 4 to 8 hours when TMA is suspected and eculizumab is considered standard care for HUS.
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