United States

In-stent thrombosis (ISR) is one of the dreaded complications of percutaneous coronary angioplasty. Despite marked reductions in ISR since the transition from bare metal stents to drug-eluting stents, ISR still occurs at a rate of 1%-2% per year. 

We present the case of a 64-year-old Caucasian male who was admitted for staged angioplasty after a left heart catheterization had shown significant stenosis to the LAD and mid ramus intermedius. He had DES placed to the mid-LAD and mid-ramus intermedius. Despite being placed on dual antiplatelet therapy after the stent placement, six hours later, he complained of chest pain and EKG showed new ST elevations in leads I, aVL, and V2-V6. Repeat LHC showed thrombosis to recently placed LAD and mid ramus-intermedius stents, so a new set of stents was placed, and the patient was loaded with eptifibatide and then restarted on dual antiplatelet therapy after the procedure.

However, only 48 hours later, the patient started complaining of chest pain again. EKG showed new ischemic changes and a high sensitivity troponin of 5000. He was taken to the catheterization lab, and LHC once again revealed late stenosis of the previously stented LAD and mid-ramus intermedius. He underwent percutaneous coronary angioplasty to these stents. Post-LHC, he was started on prasugrel and aspirin instead of the 2 other times when he was on clopidogrel and aspirin, then ticagrelor and aspirin.

In addition to the dual antiplatelet therapy, he was also started on a Factor X inhibitor, rivaroxaban. Our hypercoagulability investigations did not yield any positive results; however, we found out the patient was on testosterone replacement.

This case is very unique in that we had early re-stenosis of a drug-eluting stent, and it did not only occur once, it occurred twice. One of the questions raised in this unique case is: when you have patients who have early restenosis of their stents, what is the preferred antiplatelet agent, and is a Factor Xa inhibitor indicated in this scenario? In the case of our patient, we went for prasugrel + aspirin + rivaroxaban, and we did not have any re-stenosis.


AMA Research Challenge 2024 

Managing Recurrent Acute In-Stent Thrombosis: Insights from a Single Case Study


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Background
Vasculitides encompass a diverse group of rare inflammatory disorders that affect blood vessels, each presenting with distinct clinical and pathological characteristics. Over 30 types of vasculitides have been identified, differentiated by their patterns of vessel involvement, serological markers, and histopathological features. Accurate diagnosis is crucial for determining long-term survival and appropriate therapy, necessitating a thorough evaluation to classify the specific type of vasculitis and exclude other conditions that mimic it.
Case Presentation
We present a 59-year-old female who presented with oral mucositis, purpuric skin lesions, and bilateral cyanosis of the toes. Upon presentation, necrosis of the toes was identified, and the patient underwent ten-toe amputation. Despite the resolution of mucositis and purpuric lesions, she now presents with acute skin changes to her left index finger concerning for additional necrosis. An angiogram revealed arteriographic evidence of vasculitis in small tributaries, displaying a beaded appearance along the plantar surface of the foot. Histological examination was nonspecific, showing necrosis with inflammatory cell infiltration. A comprehensive rheumatologic workup was negative for antiphospholipid antibodies, ANCA, anti-DNA, ANA, CCP, MPO, RF, and Proteinase 3. The patient was started on steroids, which halted the progression of necrosis in her index finger. Despite an extensive serological and histopathological workup, no clear predisposing cause or diagnosis was established. The working diagnosis remained thromboangiitis obliterans, attributed to a remote history of smoking. Through her stay and discharge, the treatment team conducted patient-centered rounds by engaging in open dialogue with the patient and involving her physician acquaintances.
Discussion
With an elusive diagnosis, the type of vasculitis afflicting our patient remained unidentified. Her constellation of findings does not align with any known form of vasculitis, and the work-up provides no evidence to corroborate a specific pathology. Despite a leading diagnosis of thromboangiitis obliterans, empiric steroid therapy provided clinical benefit, which is not typical for the condition. Furthermore, the lack of a definitive diagnosis complicates prognostic assessments and the selection of concurrent immunosuppressive treatments. While our patient was equally frustrated at the lack of definitive answers regarding a diagnosis, the team's open communication fostered a relationship of trust, resulting in the patient's cooperation, thus enabling the team to stabilize our patient's symptoms in the setting of diagnostic uncertainty. Frequent updates and patient-centered rounds increased transparency about the goals of the patient and medical team. Our case demonstrates the importance of a systematic, evidence- based approach to vasculitis workup and establishing a therapeutic alliance with our patients.

Navigating Diagnostic Uncertainty in a Case of Atypical Vasculitis

Posterior reversible encephalopathy syndrome (PRES) is a clinical-radiographic diagnosis that can be visualized as symmetric posterior white matter vasogenic edema in the occipital and parietal lobes that arises as a consequence of endothelial damage in vulnerable vasculature. PRES can present clinically as visual changes, headaches, seizures, and altered mental status. Although most cases of PRES have been described in the setting of hypertension, a rare subset has developed in the setting of blood transfusion in chronic anemia.

Here, we present the first case of an adult male who developed PRES following a blood transfusion. The patient is a 69-year-old caucasian male who had an acute exacerbation of chronic anemia and went on to develop PRES following the transfusion of three units of packed red blood cells. 

21 total cases of PRES following a blood-transfusion have been documented. 20 of these cases are reported in females and 1 in an adolescent male. In the setting of chronic anemia correction in patients with identifiable risk factors (i.e., hypoalbuminemia or metabolic acidosis), care should be taken to transfuse blood products over long periods of time with close monitoring of hemoglobin levels to mitigate the risk of developing PRES. In patients with PRES, early identification and discontinuation of inciting factors are critical to a favorable prognosis.

Posterior Reversible Encephalopathy Syndrome Related to Blood Transfusion in a Male Chronic Anemic: A Case Report

Introduction:
Primary rib neoplasms are uncommon, making up only 5-10% of all bony tumors and 5% of thoracic neoplasms. Primary rib neoplasms are also not typically considered in the list of common differential diagnoses for thoracic musculoskeletal pain alongside conditions such as costochondritis. Additionally, primary rib neoplasms can present similar to other conditions, such as Tietze Syndrome. Because of the prevalence of primary rib neoplasms as well as its wide range of presenting symptoms, the time to diagnosis is typically prolonged. Primary rib neoplasms are typically treated surgically. We discuss a patient presenting with a 5-year history of intermittent left chest pain who was found to have a chondroid lesion of the left rib after years of extensive cardiac and radiologic workup. This case highlights the importance for physicians to consider primary rib neoplasms in the differential for patients with musculoskeletal chest pain.

Clinical Vignette Summary:
A 39 y/o F with a PMH of bilateral avascular necrosis of the hips presents with a 5-year history of intermittent left chest pain. Patient reports that the pain began after shutting the hatchback of a car in 2018. She notes that the pain is exacerbated by movement of her left arm and shoulder and occurs roughly once a week. Patient was initially given a diagnosis of costochondritis in 2018. However, in 2021, an EKG and Echo were ordered due to patient report of chest tightness; both tests returned within normal limits. In November 2022, extensive imaging was conducted due to persistence of pain. CT Chest w/o contrast revealed a 10 x 5 mm lesion in the left third costal cartilage with appearance of a benign chondroid lesion, corresponding with a T2 hyperintense focus on MRI of the Left Upper Extremity. Patient was sent to Cardiothoracic Surgery for evaluation and ultimately opted for conservative management with acupuncture and topical diclofenac and lidocaine due to stability of symptoms. 

Conclusions/Clinical Relevance:
This case highlights the importance of broadening one’s differential when it comes to the workup of musculoskeletal pain. While diagnoses such as costochondritis are common causes of musculoskeletal pain, quickness to diagnose patients with this condition may lead clinicians to miss other potentially more serious causes of musculoskeletal pain.

Primary Rib Neoplasms: Considerations For Providers in the Workup of Musculoskeletal Pain – A Clinical Vignette

Background

Statins, widely prescribed for cardiovascular disease, effectively reduce cardiovascular risk in primary and secondary prevention. However, muscle pain is a common side effect, affecting 2% to 11% of users. It is crucial to differentiate between benign muscle pain and serious myopathies, as severe myopathy (characterized by a creatine kinase (CK) level greater than 10 times the reference value) occurs in approximately 0.5% of patients. Immune-mediated necrotizing myopathy (IMNM), associated with anti-HMGCR antibodies, anti-SRP auto-antibodies, HIV, hepatitis C virus (HCV), connective tissue disorders, paraneoplastic disease, or idiopathic causes, is one such severe condition. IMNM presents with acute or subacute mild to moderate symmetrical muscle weakness, and discontinuing statins does not halt muscle weakness and elevated CK levels in HMGCR antibody-associated IMNM.

Case Presentation

A 67-year-old female presented with muscle weakness and elevated creatine phosphokinase (CPK) levels. Initially active, she began experiencing muscle weakness, particularly with overhead movements, in October 2023. She consulted her primary care doctor and was referred to a rheumatologist, who discontinued her statin medication in March 2024. Her symptoms temporarily improved but then worsened, with leg weakness and difficulty swallowing. By April 2024, her CPK level rose to 4806. 

Laboratory findings revealed anemia of inflammatory condition, elevated AST (175 U/L) and ALT (195 U/L) levels, a low A/G ratio, and low serum potassium. CT scans of the chest, abdomen, and pelvis with contrast did not indicate malignancy. Immunological testing revealed an elevated HMGCR Antibody IgG at 95 and SSA-52 (RO52) at 43. A muscle biopsy indicated skeletal muscle necrosis and regeneration. 

The patient received 1 gram of Solu-Medrol (Methylprednisolone sodium succinate), significantly improving her muscle strength and mobility, with CPK levels decreasing to 2779 and then to 855. The treatment plan included IV Solu-Medrol for three days, followed by oral Prednisone, Vitamin D, a proton pump inhibitor (PPI), and scheduled IVIG therapy for 2 days. Follow-up showed symptom relief and normalization of CPK levels.

Discussion

This case underscores the importance of recognizing statin-induced myopathy, particularly HMGCR antibody-mediated IMNM. Before diagnosing statin-induced myopathy, various differential diagnoses, including dermatomyositis, myopathy/myositis with significant CK elevation, polymyalgia rheumatica, and HMGCR antibody-mediated IMNM, must be considered. Understanding its natural course, particularly in cases of HMGCR antibody-mediated IMNM, is essential for informed patient consent and effective management. This case highlights the need for thorough differential diagnosis and appropriate treatment to ensure optimal patient outcomes.

Statin-Induced Myopathy Case Report

Background:
Plexiform neurofibroma is a pathognomonic manifestation of Neurofibromatosis type 1 (NF1), a rare autosomal dominant genetic disorder caused by a mutation in chromosome 17q 11.2, affecting approximately 1 in 3000 births. Elephantiasis Neuromatosa (EN) is an uncommon presentation of plexiform neurofibroma, which leads to severe limb hypertrophy and can be mistaken as Filariasis. Here, we present a challenging adult case of EN initially misdiagnosed as filariasis for 15 years. Prompt recognition and accurate diagnosis were crucial for managing rare NF-1 presentations such as EN, addressing treatment complexities, and access challenges. By navigating beyond the traditional borders of diagnosis and treatment, this case sheds light on the intricate management dilemmas faced in such scenarios.

Case Presentation: 
A 19-year-old Guatemalan male presented with long-standing left lower limb abnormalities, initially suspected as Filariasis. The patient had recently migrated from Guatemala specifically seeking treatment for Filariasis. On physical examination, pronounced enlargement of both the phallus and scrotum was noted, along with palpable cystic masses, prompting clinical suspicion of severe lymphedema secondary to Filariasis. However, further examination revealed extensive masses affecting the thigh, pelvis, genitals, and lower leg, with associated clinical features indicative of NF1. Multiple café-au-lait macules were evident over the chest, back, and torso, with larger lesions around the lower left trunk. Additionally, multiple small nodules consistent with cutaneous neurofibromas were present. MRI showed extensive fluid-filled lesions throughout the pelvis and lower extremities, indicative of plexiform neurofibroma. Biopsy revealed spindle cells positive for S100. Genetic analysis revealed a pathogenic variant within the NF1 gene, confirming the diagnosis of NF1. Treatment with Selumetinib, a selective MEK inhibitor, was considered. However, Trametinib was initiated due to insurance constraints. Remarkably, significant improvement was observed after one month of treatment.
Discussion: 
The case highlights the diagnostic challenges encountered in identifying EN, particularly when it presents as Filariasis. NF-1 diagnostic criteria, including characteristic clinical features and genetic testing, play a vital role in accurate diagnosis. Imaging modalities such as MRI aid in confirming the diagnosis by revealing typical features of plexiform neurofibroma. Treatment options for EN, primarily surgical intervention, pose significant risks due to high risk of bleeding and the potential for recurrence. Although Selumetinib is approved only for pediatric population, Trametinib is a viable alternative as well, demonstrating its efficacy in improving symptoms.

Tackling Elephantiasis Neuromatosa Presented as Filariasis in an Adult - A Management Dilemma

Abstract Title 

Teach Back, Don't Come Back: Reducing Readmissions Through Effective Discharge Communication

Background
Patient and caregiver education at the time of hospital discharge is critical transitioning care, but is often ineffective. Barriers to discharge communication can include diminished functional capacity due to illness, socioeconomic factors such as inability to afford new medications, limited understanding of the expected progression of symptoms, and schedule incompatibility between patient caregivers and hospital staff. Increasing the quality of communication between physician teams and patient families at the time of discharge has the potential to improve patient outcomes and reduce readmissions.

Case Presentation
Francesca (pseudonym) is an elderly woman with a history of stroke 15 years prior with residual speech and motor deficits. She is non-verbal and is cared for by her two daughters. She has presented to the emergency department several times in the past two weeks for altered mental status, and was incidentally found to have a positive urinalysis, which was treated with antibiotics 1 week prior.

She is brought by her daughter today due to concern for altered mental status. Physical exam is consistent with her documented baseline since the stroke. A review of her medical history shows a pattern of hospital admissions for non-specific mental status changes over the past 6-7 years and a primary care note documenting a diagnosis of vascular dementia 8 years prior. The team concludes that Francesca’s family may be interpreting fluctuations in her status as new illness, when they are more likely chronic sequelae of her underlying dementia.

The treating team briefly met with one of Francesca’s daughters during morning rounds, and explained that her symptoms were consistent with vascular dementia. She was discharged later that day. 

One month later, Francesca returned with both daughters due to mental status changes. Examination and imaging revealed no changes. One daughter recalled the prior admission, stating “When she was here one month ago, the doctors mentioned that this might be her new normal. I’m not really sure how to tell whether this is normal, or whether something concerning is going on.”

Discussion
This case illustrates many challenges of effective discharge communication: only one of the patient’s caregivers was present, the conversation was rushed due to morning rounds, and communication was delivered primarily by the physician, with little time devoted to soliciting the family’s concerns or questions. In this instance, the family’s awareness of the expected course of symptoms was incomplete, and resulted in an unnecessary readmission.


Teach Back, Don't Come Back: Reducing Readmissions Through Effective Discharge Communication

Background:
Diseases in elderly populations are commonly multifactorial due to multiple comorbidities and sedentary lifestyles. Proteus mirabilis is a highly motile opportunistic gram-negative facultative anaerobe in the intestinal tract. It primarily affects the urinary tract, but is also a rare cause of pneumonia seen in older patients with chronic lung disease and, occasionally, pericardial effusion. We present a case of an elderly male with hypoxia found to have pericardial effusion, lung atelectasis with proteus pneumonia, and recurrent sigmoid volvulus leading to multiple emergency department (ED) visits.

Case Presentation: 
A 68-year-old male with a history of smoking, chronic obstructive pulmonary disease (COPD), asthma, venous stasis, aspiration pneumonia, respiratory failure and recurrent sigmoid volvulus was brought to the ED from his assisted living facility with SpO2 of 80%, pH of 7.09, and pCO2 of 132.8 mmHg. Patient received supplemental oxygen and nebulizer treatment and further workup was done. Complete blood count showed red and white blood cells low at 3.97 and 3.63, respectively. CT with contrast of chest and abdomen showed left mainstem bronchus mucus plug with atelectasis and recurrent sigmoid volvulus. An urgent decompressive colonoscopy resolved his volvulus. Patient had severe respiratory acidosis post-op that required intubation given a failed bilevel positive airway trial. 

The patient had a cardiac arrest the following day and was resuscitated and emergent bedside pericardiocentesis for pericardial effusions causing cardiac tamponade (evident on emergent echocardiogram). The pericardial drain remained in place for 7 days due to continued high volume drainage (>80mL/day). Additionally, a bronchoscopy successfully removed the left bronchus mucus plug and a bronchoalveolar lavage in the left lower base showed proteus pneumonia. Cefepime was initially administered and then de-escalated to ceftriaxone 3 days later. Patient was discharged to a hospice facility to continue care. 


Discussion:
Multiorgan involvement can lead to poor prognosis given complexities of each organ system and concern of side effects from management of other systems. The symptoms of pericardial effusion and atelectasis were masked by the most obvious sigmoid volvulus on imaging. The multifactorial etiologies of hypoxia should be considered and appropriate workup should be done in a timely fashion. 

This case illustrates the importance of comprehensive workup in older populations with multiorgan diseases. Those populations with limited mobility are at significant risk of several multifactorial comorbidities that can lead to life-threatening emergencies without prompt and appropriate interventions.


The Red Herring Sigmoid Volvulus: Stealing Attention from Pericardial Effusion and Proteus Pneumonia

Introduction:

Nocardia are soil-borne, aerobic microorganisms, which are often causes of opportunistic infections in immunocompromised patients. There are 92 identified Nocardia species out of which 54 of them have been identified to infect humans, Nocardia beijingenesis being one of them. It can manifest as pulmonary, disseminated, or cutaneous infection. Early diagnosis and prompt treatment are vital in the overall prognosis of patients. We present a case of a young male with HIV who had Nocardiosis without travel history outside of the United States.

Case description:

A 37-year-old man, diagnosed with HIV two years ago and currently not on antiretroviral therapy, presented with flu-like symptoms and weight loss over the last two months and confusion for two days. The patient denied any international travel outside the United States. On presentation, although hemodynamically stable, he was found to have severe hyperkalemia, metabolic acidosis, and acute kidney injury. The patient underwent emergent hemodialysis. CT scan of the lungs without contrast showed multifocal pneumonia, suspicious for necrotizing pneumonia on the left with dense consolidation, cavitation, and air bronchograms with mild involvement of the right lung. CT head and MRI brain did not reveal any intracranial abnormalities. Imipenem-cilastin and minocycline were started initially along with atovaquone for Pneumocystis jiroveci pneumonia prophylaxis. Although blood cultures were negative, the aspergillus galactomannan assay was positive. Next-generation sequencing as well as the culture of bronchoalveolar lavage detected Nocardia beijingensis. The antibiotics were later tailored to ceftriaxone, minocycline, and linezolid to be continued for a minimum of three months, followed by monotherapy for at least six months which is to be guided by susceptibilities results in the outpatient setting. 

Discussion:

Nocardia, an aerobic, weakly gram-positive opportunistic bacteria, primarily affects immunocompromised individuals, including those with HIV. One of its less common species, Nocardia beijingensis, was initially reported in the US in 2014. This infection may present as pulmonary, cutaneous, or disseminated nocardiosis. Pulmonary nocardiosis, the most common form, manifests with chest pain, coughing, sputum production, and sometimes hemoptysis resembling tuberculosis, aspergillosis, or bacterial pneumonia. Skin infections by Nocardia beijingensis may present with cellulitis, draining lesions, or nodules. Dissemination occurs in about 45% of cases, commonly via the bloodstream, affecting various organs. The central nervous system is often involved, that presents with headaches, nausea, altered mental status, and seizures. Nocardia beijingensis, due to diagnostic complexities, is associated with numerous

complications, notably abscesses in the lungs, skin, brain, adrenal glands, subretinal space, and muscles. It can also lead to rare complications like superior vena cava syndrome and cardiac tamponade. Due to its high mortality rate, early diagnosis is crucial but challenging due to nonspecific symptoms. Diagnosis involves gram staining, modified acid-fast staining, and cultures from clinical specimens. Advanced molecular techniques such as gene sequencing of the 16SrRNA, PCR, and restriction gene analysis are also commonly used for species identification. Although trimethoprim-sulfamethoxazole (TMP-SMX) is a common treatment, resistance has led to a preference for combination therapy, especially in severe, disseminated cases, or those with central nervous system involvement. Antibiotic susceptibilities vary among Nocardia species, with many responsive to TMP-SMX, ceftriaxone, minocycline, and linezolid. The duration of treatment, usually lasting 6 to 12 months is determined on expert recommendations as there are no trials establishing a definitive timeframe. TMP-SMX also offers prophylaxis to Nocardia infections which is beneficial as it is commonly used in HIV patients for prophylaxis of pneumocystis infections. 

The unexpected arrival: Nocardia Beijingensis in a patient with no travel history​

The Uncommon Journey of a Jaundiced Patient
Ashley Dababneh DO PGY2, Radhika Garimella MD PGY1, David Minter DO

Stauffer syndrome is an uncommon paraneoplastic syndrome associated with renal cell carcinoma and is characterized by hepatic dysfunction without metastasis. Due to the rarity of its occurrence, this case should be reported to broaden a physician's differential diagnosis for hyperbilirubinemia and jaundice to correctly manage the patient. A 55-year-old male with past medical history of traumatic brain injury,cerebrovascular accident with residual left-sided weakness and seizure disorder was sent to the emergency department from his assisted living facility due to 5 days of jaundice and markedly elevated bilirubin discovered by his primary physician. Patient denied any symptomology. The physical exam was significant for jaundice and scleral icterus. Initial labs in the ER showed total bilirubin of 8.7 with a direct bilirubin of 6.67 and indirect bilirubin of 2.0 and alkaline phosphatase was 540. Computed tomography of the abdomen/pelvis demonstrated mild gallbladder thickening and intrahepatic biliary dilatation with cholelithiasis and common bile duct prominence with no obvious obstructing stone or mass. It also showed signs consistent with low-grade colitis and a mass-like attenuation of the right kidney. Ultrasound of the abdomen displayed non-specific gallbladder distention, gallstones and biliary sludge without wall thickening. Gastroenterology was consulted and further investigation showed normal gamma glutamic transferase, carbohydrate antigen 19–9, antimitochondrial antibody, and smooth muscle antibody levels. Magnetic resonance cholangiopancreatography showed a distended gallbladder with gallstones and choledocholithiasis. It also displayed a mixed signal mass measuring 3.3 X 3.8 X 3.3 cm at the mid pole of the right kidney concerning for a neoplastic process. Endoscopic retrograde cholangiopancreatography was completed, removing an obstructing gallstone and sphincterotomy was performed. The patient was scheduled for cholecystectomy after the obstructing gallstone was removed, however, due to the rising direct bilirubin, alkaline phosphatase and repeat imaging showed no signs of filling defects, the surgery was postponed. The renal mass is initially planned to be followed up outpatient, but urology was consulted due to the concern for possible Stauffer syndrome and the patient underwent a right nephrectomy. Throughout the remainder of his hospitalization his bilirubin trended down leading to improvement of his jaundice and his pathology report showed clear-cell renal carcinoma. 
This case demonstrates the need to expand was differential diagnosis and to not exclude incidental findings on imaging. The jaundice variant presentation of the syndrome is rare, but a necessary addition to the differential the patient with a renal mass concerning for neoplasm and hyperbilirubinemia.

The Unexpected Journey of a Jaundiced Patient

These Antibiotics Just Aren’t Working: Fever of Unknown Origin and an Uncommon Culprit

Kajol Patel MD, WellStar MCG Health Department of Neurology; Nicholas Fuchs BBA Medical College of Georgia; Alexander Warner MD, WellStar MCG Health Department of Internal Medicine; Derek Backer MD MPH, WellStar MCG Health Department of Internal Medicine

Background: Fever of unknown origin (FUO) is defined as a periodic temperature of at least 38.4°C lasting longer than 3 weeks, with a diagnosis remaining uncertain after 1 week of evaluation. The potential etiologies of FUO are diverse, which adds to the challenge of determining the cause. We report a case of FUO that ultimately led to the diagnosis of a very rare cause of fever in adults: Adult-onset Still’s disease (AOSD). Cases like this pose a diagnostic dilemma due to the hesitancy to withhold antimicrobials in the presence of a persistent fever. However, despite the lack of an accepted standard workup for FUO, a thorough and methodical evaluation increases the likelihood of arriving at a treatable diagnosis.

Case Description: A 41-year-old female with a past medical history of psoriatic arthritis presented to the hospital after more than 2 weeks of undulating fevers and bilateral leg pain. She has a past history of psoriatic arthritis and a remote history of IV drug use reportedly in remission. She had previously been treated with antibiotics but no source was clearly identified. Her review of systems included pertinent positives such as odynophagia, salmon colored rash which worsened with the fevers, polyarthralgia, and negatives such as lack of cough or shortness of breath, diarrhea, or dysuria. She had a neutrophilic leukocytosis, markedly elevated CRP and ESR, and markedly elevated ferritin. After ruling out alternatives such as bacteremia, endocarditis, occult abscess, thrombosis or other connective tissue diseases, it was noted that the patient fulfilled 3 major and 4 minor Yamaguchi criteria and the diagnosis of Adult-onset Still’s Disease was made.

Case Discussion: Adult-onset Still’s disease is a very rare cause of fever of unknown origin. Its symptoms include inflammatory arthralgia, a salmon-colored rash, sore throat, lymphadenopathy, and hepatosplenomegaly. AOSD is estimated to occur in 0.16-0.4 per 100,000 persons. Due to its rarity and symptom overlap with other conditions, diagnosing AOSD can often take several months.
This case highlights AOSD as a rare but debilitating cause of FUO. If left untreated, AOSD can lead to significant morbidity and irreversible organ damage. Our case demonstrates the importance of conducting an exhaustive evaluation for alternative causes of FUO before initiating steroids or disease-modifying antirheumatic drugs. When the correct therapy is initiated, the response can be both dramatic and rewarding.

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