RARE PRESENTATION OF LUPUS: SLE PRESENTING AS SEPTIC SHOCK

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.https://scholarlycommons.henryford.com/merf2020caserpt/1040/thumbnail.jp

The Canary in the Coal Mine

Case: The patient is a 32-year-old Asian male with PMH of ERSD secondary to minimal change disease (diagnosed at age 26) presented for acute onset shortness of breath, lower extremity edema, and abdominal distension. He underwent CTPE which showed large left pleural effusion, moderate pericardial effusion and as well as ascites. Transthoracic ECHO showed moderate to large pericardial effusion with evidence of tamponade physiology. The patient underwent emergent pericardiocentesis and placement of a pericardial drain. The pericardial fluid was bloody in appearance, fluid studies showed RBC 2047709, cytology negative malignancy and culture showing no growth. The patient also underwent left-sided thoracentesis with the removal of 1L bloody fluid with the placement of a left-sided pleural drain. Pleural fluid studies showed RBC 30803, cytology negative for malignancy and culture showing no growth. Furthermore, paracentesis was done with the removal of 800cc peritoneal fluid with bloody appearance. Fluid studies showed RBC 112577 and cytology negative for malignancy, negative cultures. Hemoglobin was around 8, platelets 102, CRP 18 and sed rate 53. ANA 1:640 speckled pattern, low C3 and C4, negative ANCA, RF, CCP, RNP, Smith ds DNA, SSA, SSB, C3 low and C4 normal (both initially low). He met 4/11 SLICC criteria for SLE including serositis, +ANA, anemia/thrombocytopenia and low complements, history of MCD. He was started on methylprednisolone resulting in the improvement of anemia/thrombocytopenia. Then transitioned to oral prednisone with a taper and was also started on plaquenil and imuran. Impact/Discussion: To date, the association of SLE with MCD has been described only in isolated case reports. Initially, the etiology of the patient’s MCD was unclear. He had no history of lupus nephritis and no known causes for secondary FSGS, but failed to recover renal function and ultimately progressed to ESRD. His dramatic presentation suggests that MCD was a harbinger of SLE—the metaphorical canary in the coal mine. In the context of his past medical history and his particular demographic, initial clinical suspicion for SLE was low. This case contributes to the body of literature supporting early consideration of SLE as a potential etiology for MCD and acute pericarditis. Conclusion: SLE is an autoimmune disorder with a wide spectrum of manifestations. Acute pericarditis is an important and potentially a life threatening complication of the disease. According to the most recent EULAR/ACR 2019 SLE criteria, acute pericarditis scores 6 points. A total of 10 points is required to diagnose SLE, thus recognizing SLE as a potential underlying cause in a newly diagnosed acute pericarditis or cardiac tamponade is crucial to initiate treatment and improve outcomes. A relevant association may exist between SLE and MCD.https://scholarlycommons.henryford.com/merf2020caserpt/1019/thumbnail.jp

LADA presenting as hyperglycemic coma

Latent Autoimmune Diabetes in Adults (LADA), is considered a rare subset of Type 1 Diabetes Mellitus. These adults do not require insulin at diagnosis but progress to insulin dependence in a short period of time. Because of this the diagnosis is often missed and can result in potentially fatal complications such as Diabetic Ketoacidosis (DKA. Our case highlights one such example with extreme levels of hyperglycemia of 2345mg/dl and its unique therapeutic challenges. A 49-year-old male with a strong family history of diabetes and recent diagnosis of prediabetes in the outpatient setting was found unresponsive at home. He had profound hypothermia 28.4 C, hyperglycemia 2345mg/dl, corrected sodium 182mg/dl and ketoacidosis. Due to altered mental status and seizures he was intubated and fluid resuscitated. Nephrology were consulted due to high risk of cerebral edema with rapid correction, they advised lowering of glucose no more than 50mg/dl/hour. He was started on a customized DKA protocol and his mentation and all laboratory abnormalities improved over a 72 hour period. He was extubated and transferred to the floor. Further workup revealed a C-peptide level of 120 IU/ml. Patient was diagnosed with Type 1 Diabetes Mellitus (LADA).Type 1 diabetes mellitus is often unrecognized until acute decompensation. This case highlights the importance of remaining vigilant outside of the typical adolescent age group. In addition, it describes complications of extreme hyperglycemia including encephalopathy, hypothermia, seizures, acute kidney injury and its careful management in order to prevent catastrophic fluid shifts.https://scholarlycommons.henryford.com/merf2020caserpt/1114/thumbnail.jp

EHR Visual Overlay Promises to Improve Hypertension Guideline Implementation

Background: Primary care management of essential hypertension (HTN) has become increasingly challenging since recently published guidelines integrate atherosclerotic cardiovascular disease (ASCVD) risk stratification into decision making. Our objective was to measure whether overlay of visualdecision support (VDS) with standard electronic health record (EHR) platform improves guideline-based treatment, and reduces time burden associated with EHR use, in management of essential HTN. Methods: This was a quality improvement project. We interviewed primary care physicians and tasked each with two simulated patient encounters for HTN: (1) using standard EHR to guide treatment, and (2) using VDS to guide treatment. The VDS included graphical blood pressure (BP) trends, target BP with recommended interventions, ASCVD risk score, and information on the patient’s social determinants of health. We assessed whether treatment selection was congruent with guidelines and tracked time physicians consulted the EHR. Results: We evaluated 70 case simulations in total. Use of VDS compared to usual EHR was associated with: higher proportion of correct guideline prescribing (94% vs. 60%, p\u3c0.01), more ASCVD risk determination (100% vs. 23, p\u3c0.01), and more correct BP target identification (97% vs. 60%, p\u3c0.01). Time clinicians spent consulting the EHR fell an average of 121 seconds with use of VDS (p\u3c0.01). On a 10-point scale, clinicians rated the VDS 9.2 vs. 5.9 (p\u3c0.01) for ease of gathering necessary information to treat HTN. Conclusions: The integration video decision support tools to standard EHR can reduce physician time spent per patient encounter, while increasing adherence to guidelines and improving patient outcomes. Further testing in clinical practice is indicated.https://scholarlycommons.henryford.com/merf2019qi/1009/thumbnail.jp

Visual Analytics Dashboard Promises to Improve Hypertension Guideline Implementation

BACKGROUND: Primary care management of hypertension under new guidelines incorporates assessment of cardiovascular disease risk and commonly requires review of electronic health record (EHR) data. Visual analytics can streamline the review of complex data and may lessen the burden clinicians face using the EHR. This study sought to assess the utility of a visual analytics dashboard in addition to EHR in managing hypertension in a primary care setting. METHODS: Primary care physicians within an urban, academic internal medicine clinic were tasked with performing two simulated patient encounters for HTN management: the first using standard EHR, and the second using EHR paired with a visual dashboard. The dashboard included graphical blood pressure trends with guideline-directed targets, calculated ASCVD risk score, and relevant medications. Guideline-appropriate antihypertensive prescribing, correct target blood pressure goal, and total encounter time were assessed. RESULTS: We evaluated 70 case simulations. Use of the dashboard with the EHR compared to use of the EHR alone was associated with greater adherence to prescribing guidelines (95% vs. 62%, p\u3c0.001) and more correct identification of BP target (95% vs. 57%, p\u3c0.01). Total encounter time fell an average of 121 seconds (95% CI 69 - 157 seconds, p\u3c0.001) in encounters that used the dashboard combined with the EHR. CONCLUSIONS: The integration of a hypertension-specific visual analytics dashboard with EHR demonstrates the potential to reduce time and improve hypertension guideline implementation. Further widespread testing in clinical practice is warranted

Pyeloduodenal Fistula: A Rare Complication

Objectives: Diagnose pyeloduodenal fistulaManage pyeloduodenal fistulaCase: 85-year-old male with a history of bladder cancer status post tumor resection and left ureteral stent placement presented with right flank pain, hematuria, and CVA tenderness on physical exam. UA was consistent with urinary tract infection, CT abdomen pelvis showed gas and fluid in the dilated right renal pelvis and collecting system. Cystoscopy with bilateral retrograde pyelogram was performed, but urologists were unable to place right stent due to stricture and unable to remove left stent. Bilateral nephrostomy tubes were placed, but a fistulous connection was noted between the right collecting system and the duodenum. Upper GI and small bowel follow-through showed opacification of the right intrarenal collecting system, consistent with a fistula between the duodenum and the right renal collecting system. EGD showed an ulcer in the duodenum with a small central fistula, correlating with the known pyeloduodenal fistula. This was treated with argon plasma coagulation and closed using the Tulip Bundle method of placing hemoclips around the margin and closure with an endoloop around the clips. Discussion: A pyeloduodenal fistula is a rare but serious condition, generally associated with chronic inflammatory renal diseases or trauma.The posterior aspect of the second portion of the duodenum lies in proximity to the medial portion of the kidney and renal pelvis. When perirenal inflammation takes place, this portion of the duodenum is more easily involved. Pyeloenteric fistulas may be classified as traumatic or spontaneous, which constitute the majority of cases. Malignant etiologies include urothelial carcinoma, renal transitional cell carcinoma and nephrolithiasis-related squamous cell carcinoma and adenocarcinoma. Diagnosis of pyeloduodenal fistula requires imaging studies of the urinary system. Retrograde pyelography is the method of choice which demonstrates the fistula in 64% of cases. Intravenous urography can be used only in functioning kidneys, and CT often shows fistula. Other imaging modalities include antegrade pyelography, EGD and upper GI studies.Nephrectomy and primary closure of the duodenum are traditional treatment methods. Small sized fistula and accessible location by EGD may be favorable features for nonsurgical treatment. Nonsurgical management includes intravenous antibiotics, relieving obstruction by employing nephrostomy, or internal ureteric stents. A new method of treatment is endoscopic ligation, as was seen in this case. Conclusion: Retrograde pyelography is the study of choice for diagnosing pyeloduodenal fistula in a functioning kidney. EGD can be used both for diagnosis and intervention. Conservative management with antibiotics and stent placement may be possible with small fistulas. Endoscopic intervention by an advanced GI team can be done to avoid extensive surgical intervention.https://scholarlycommons.henryford.com/merf2019caserpt/1047/thumbnail.jp

Small cell lung carcinoma with three paraneoplastic syndromes in one patient

SESSION TITLE: Monday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/21/2019 02:30 PM - 03:15 PM INTRODUCTION: This is a case of patient who was diagnosed with Small Cell Lung Carcinoma(SCLC)after presenting with symptoms of Dermatomyositis(DM), SIADH and Lambert–Eaton myasthenic(LEMS)syndrome CASE PRESENTATION: Patient initially presented to the clinic with erythematous patches scattered on the trunk, extremities, and scalp associated with pain, itchiness and hair loss for two months. Biopsy from rash showed perifollicular and mild superficial perivascular inflammation. The patient was treated for viral exanthem with steroids. Patient subsequently presented to the hospital with worsening pruritic patches on scalp and entire body and periorbital edema. This was associated with weakness and generalized malaise. The weakness and pain were worse with neck and shoulder movements and the weakness improved on exertion. Other symptoms were productive cough, rhinorrhea, nasal congestion and sore throat for a month associated with 20 pounds weight loss. Physical exam showed decreased breath sounds in the left upper and middle field.No lower extremity swelling.Skin findings were violaceous edematous periorbital erythema(heliotrope rash), erythema of neck, upper chest, and entire back, erythematous slightly scaly plaques overlying MCPs and PIPs(Gottron\u27s papules). Labs showed CPK of 868, Aldolase of 8.9, serum sodium of 115(Serum osmolality 248, Urine osmolality 497, TSH 1.7, random cortisol 20.7)and ANA(1:320)was positive. Chest x-ray showed left upper lobe mass. CT chest showed malignant process arising from the left upper lobe and to the mediastinum. She underwent bronchoscopy with FNA and endobronchial biopsy which showed SCLC. DISCUSSION: Paraneoplastic syndromes occur in approximately 10% of patients with lung cancer(1). SIADH occurs in 15% of SCLC patients. LEMS is present in approximately 1% to 3% of patients with SCLC. Off all cases of DM only 5.9% have lung cancer. In some cases these syndromes can be presenting sign of cancer. The early recognition of paraneoplastic syndromes may contribute to the detection of a highly treatable, early-stage tumor. At other times, the syndromes may occur late in the course of disease or may appear as the first sign of recurrence DM is associated with poor prognosis and improved SCLC tumor survival are seen in patients with LEMS(2). Management of hyponatremia is very important as it is associated with a poor prognosis regardless of an extensive or limited stage. SCLC patients with serum sodium less than 129 mmol/l had a median survival of 8.63 months compared to 13.6 months in patients with normal sodium, and the degree of hyponatremia is a significant predictor for prognosis(3). CONCLUSIONS: SCLC is associated with large number of paraneoplastic syndromes which can also be the initial presentation in some cases. Ability to identify these syndromes especially in high risk population can lead to early diagnosis and might carry a better prognosis in some patients

A rare case of spontaneous asymptomatic ventricular tachycardia due to arrhythmogenic right ventricular dysplasia (ARVD)

Background Arrhythmogenic right ventricular dysplasia (ARVD) is an inherited cardiomyopathy that can lead to heart failure and sudden cardiac death. This challenging diagnosis is based on clinical, electrocardiographic, and radiographic findings, along with suggestive genetic testing. Case A 72 year old man presented with stable ventricular tachycardia. 2D echo demonstrated an EF of 45%, grade I diastolic dysfunction, and LV hypokinesis. Left heart catheterization revealed non-obstructive coronary disease. Cardiac MRI revealed: dilated RV with basal RV wall akinesis; RV end-diastolic volume index 134 mL/m2, global LV hypokinesis, an area of mid-myocardial delayed gadolinium enhancement of the mid-inferoseptal LV myocardium at the RV insertion point, and no associated lymphadenopathy. Subsequent Invitae genetic testing revealed desmoplakin (DSP) gene mutation. The patient was managed with AICD placement and sotolol initiation. Decision-making In this rare case of asymptomatic stable VT, cardiac MRI findings met major criteria for ARVD. The differential diagnosis included cardiac sarcoidosis, however contrast enhanced CT imaging did not reveal hilar lymphadenopathy, and cardiac MRI was unimpressive for sarcoidosis. Furthermore, DSP gene mutation is associated with autosomal dominant ARVD. Conclusion The diagnosis of ARVD can be determined with cardiac imaging and genetic testing. It is important to rule out similar diagnoses by looking for differentiating features, such as hilar lymphadenopathy and myocardial granulomatous disease which is frequently seen in cardiac sarcoidosis, but not in ARVD

Stable isotopic investigation of <i>Porites</i> coral from the Minicoy Island

1465-1470Stable isotope analysis has been performed on a coral collected from the Minicoy Island to investigate its potential as a proxy for southwest monsoonal variability. The X-Ray picture of the porites coral collected revealed about 24 annual bands (1989-2013). The mean annual extension over this period is ca. 7.7mm/year. Stable carbon (δ13C) and oxygen isotope (δ18O) analysis of these bands reveal that the isotopic composition is controlled by kinetic fractionation. There is a drop of δ18O, ca 1‰ relative to the mean value -during -1998 indicating anomalous warming of the sea surface warming

Novel Minimally Invasive Multi-Modality Monitoring Modalities in Neurocritical Care

Elevated intracranial pressure (ICP) following brain injury contributes to poor outcomes for patients, primarily by reducing the caliber of cerebral vasculature, and thereby reducing cerebral blood flow. Careful monitoring of ICP is critical in these patients in order to determine prognosis, implement treatment when ICP becomes elevated, and to judge responsiveness to treatment. Currently, the gold standard for monitoring is invasive pressure transducers, usually an intraventricular monitor, which presents significant risk of infection and hemorrhage. These risks made discovering non-invasive methods for monitoring ICP and cerebral perfusion a priority for researchers. Herein we sought to review recent publications on novel minimally invasive multi-modality monitoring techniques that provide surrogate data on ICP, cerebral oxygenation, metabolism and blood flow. While limitations in various forms preclude them from supplanting the use of invasive monitors, these modalities represent useful screening tools within our armamentarium that may be invaluable when the risks of invasive monitoring outweigh the associated benefits