INTRODUCTION:
Drug reaction with eosinophilia and systemic symptoms (DRESS) is considered a severe idiosyncratic drug hypersensitivity which can be fatal if not promptly diagnosed and treated. Here, we describe a case of hepatic sarcoidosis and autoimmune hepatitis (AIH) overlap syndrome who was found to have DRESS from taking azathioprine (AZA).
CASE DESCRIPTION/METHODS:
A 63-year-old African-American female with a diagnosis of AIH and hepatic sarcoidosis overlap based on liver biopsy (chronic hepatitis with moderate activity (grade 2/4), mild fibrosis (stage 1/4), non-caseating granulomata, and interface hepatitis with dense lymphoplasmacytic infiltration) (Figure 1), positive ANA (1:160), and elevated IgG (2140) was started on treatment with AZA and budesonide (intolerance to prednisone). She had normal TPMT activity. Patient stopped taking AZA few days later because of feeling “jittery.” She presented to the ED five weeks later with lip swelling and desquamating rash and was discharged with antihistamines. She returned to the ED two days later with worsening rash, fever, and dyspnea. Labs showed leukocytosis, eosinophilia (56.1%, 13,400/mm3), acute hepatitis, acute kidney injury, and respiratory distress with concern for DRESS. Her initial LFTs were ALT 96, AST 65, and Alk Phos 633, which were increased from her baseline. Imaging ruled out biliary obstruction and bone marrow biopsy was negative for myeloproliferative disorders such as hypereosinophilic syndrome. She was diagnosed with DRESS secondary to AZA and was treated with a short course of prednisone with transition to budesonide monotherapy. Eosinophilia and other organ dysfunction resolved. Her most recent LFTs are ALT 37, AST 42, and Alk Phos 277.
DISCUSSION:
While AZA hypersensitivity syndrome is a well-known disorder presenting with drug eruption and flu-like symptoms, to our knowledge this is the first case of DRESS secondary to AZA. Additionally, this occurred in the setting of hepatic sarcoidosis and AIH overlap syndrome which itself is a rare entity. DRESS is characterized by a long latency time between exposure and disease (2-8 weeks) and presents with end organ dysfunction requiring steroid treatment. Based on the European Registry of Severe Cutaneous Adverse Reactions (RegiSCAR) scoring system, our patient's score was 7, where a score of 6 or higher is considered definite for DRESS. This case also stresses the value of budesonide monotherapy for treating AIH and hepatic sarcoidosis overlap when other first line medications cannot be used.
Introduction: Iron deficiency anemia (IDA) is common in the hospital setting and is frequently caused by gastrointestinal bleeding (GIB). While treatment is aimed at the underlying cause of IDA, it is also targeted at replenishing iron stores to reduce anemia progression and decrease the risk of end organ damage. Objective: The primary aim of this study was to assess whether patients hospitalized with IDA secondary to GIB received iron supplementation upon discharge. Methods: Retrospective analysis evaluating 992 patients admitted to our tertiary care hospital from 1/1/17-12/1/17 who underwent endoscopy for GIB. IDA was defined as ferritin ≤ 30, iron saturation ≤ 10% or a ≥ 2gram drop from the patient's baseline hemoglobin. A secondary endpoint assessed readmission rates within a 180-day follow up period after discharge and associated morbidity and mortality. Results: A total of 228 patients met inclusion criteria for analysis. There were 115 males (50.4%) with an overall mean age of 62.6± 2.1y. Overall, 106 (46.5%) patients eligible by our criteria to receive iron therapy were discharged on this therapy. In regression analysis, receiving iron (OR = 13.6; 95% CI, 7.0-26.6) or a blood transfusion (2.72; 1.1-6.9) during hospitalization were independently associated with discharge on iron. Discharge on iron was not associated with readmission but there was a non-significant trend toward anemia improvement for those supplemented with iron. Conclusions: At our institution, physicians failed to provide iron supplementation on discharge to over 50% of eligible patients. Patients diagnosed with IDA secondary to GIB are more likely to be discharged with iron supplementation if it was started during hospitalization or they received a blood transfusion. We believe the medication reconciliation performed automatically by the electronic medical record (EMR) at the time of discharge had a significant impact on this finding.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.