United States

Background
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs) are a group of autoimmune disorders that result in systemic inflammation with distinct clinical manifestations. These further categorize this pathology into three unique subtypes. First, Granulomatosis with Polyangiitis (GPA) (previously Wegener&#39;s Granulomatosis) is associated with the PR3–ANCA autoantibody and is known for its inflammatory destruction of upper and lower respiratory tracts, as well as renal small vessels. Second, Microscopic Polyangiitis (MPA) is another type of AAV that may present similarly to GPA; however, distinct features that separate this subtype include the absence of upper respiratory involvement and the MPO–ANCA antibody predominance. Finally, Eosinophilic Granulomatosis with Polyangiitis (EGPA) (previously Churg-Strauss syndrome) is also associated with MPO-ANCA. However, this rare type of AAV presents with asthma and eosinophilia.  The pathogenesis of all three types of AAVs includes destructive inflammation that begins intravascularly and progresses to tissue destruction and end-organ damage. Early management strategies typically involve glucocorticosteroids, and rituximab and cyclophosphamide are frequently included in patients with severe disease. 

Case Presentation
Here, we present a clinical case of MPO–ANCA positive vasculitis with a benign presentation of epigastric pain and poor appetite that rapidly progresses into systemic inflammation with pulmonary and renal manifestations. In this case, an 81-year-old female with no known medical history presents with mild chronic epigastric pain and poor appetite. Clinically, the patient was stable and well-appearing, with no apparent signs of deterioration at the bedside. Early laboratory investigations were consistent with mild anemia and chronic kidney disease. Lung imaging demonstrated diffuse airspace opacities and tree-in-bud pulmonary nodules.  Screening for infectious etiologies, including bacterial (including tuberculosis), viral, and fungal, were negative. As inflammatory markers continued to uptrend, secondary inflammatory etiologies were explored, resulting in the discovery of a positive MPO–ANCA autoantibody test.  This was followed by rapid deterioration into acute hypoxemic respiratory failure, requiring mechanical ventilation, and complicated by diffuse alveolar hemorrhage, in addition to severe anemia requiring multiple transfusions and acute kidney failure.

Discussion
This clinical case highlights the rapidly progressing nature of AAVs and the frequent benign presentations that can masquerade as this aggressive pathology. As the literature continues to evolve and offer recommendations on the management of these rare autoimmune diseases, early recognition, diagnosis, and intervention are essential to minimize mortality and morbidity rates and offer patients a relapse-free prognosis and improved clinical outcomes.


AMA Research Challenge 2024 

A Rapidly Progressing Case in an 81-year old Female

Welcome to the largest national, multi-specialty research event **featuring research from 750 medical students, residents and fellows, and international medical graduates.**


#### Event Details
The 2022 American Medical Association Research Challenge includes a poster symposium hall and a semifinals hall with the top scored research. Log in at any time throughout the event to:
* **View posters and presentations** on a variety of topics and specialties
* **Score posters in the semifinals** to help decide the top five finalists (AMA member exclusive opportunity)
* **Connect with participants** and provide feedback to help advance their research

At the conclusion of this event, the AMA will announce the top five finalists who will advance to the AMA Research Challenge finals, present their research to an elite panel of judges and compete to win a $10,000 grand prize sponsored by Laurel Road.
 

#### Attend Live Forums
**Oct. 20, 6 — 7 p.m. Central**
 **How to plan a career in academic medicine** 
 Learn how to plan a career in academic medicine and get your questions answered. [Access Here](https://underline.io/events/363/lounge)
 
 **Speakers**
![](https://assets.underline.io/uploads/markdown_image/1/image/d5adb133dfc58eb0a6d469e9cedcd5d4.jpg)
Ricardo Correa, MD, EdD
![](https://assets.underline.io/uploads/markdown_image/1/image/5f9437ab87861538bf02b9ca7c0d926e.jpg)
Stuart M. Greenstein, MD
![](https://assets.underline.io/uploads/markdown_image/1/image/05b1b637c99286d558c371f8e8b185ce.jpg)
Stephanie Strohbeen, MD
 
 **Oct. 22, 11 a.m. — Noon Central**
 **How to get published**
 Learn tips to get your research published and get your questions answered. [Access Here](https://underline.io/events/363/lounge)
 
 **Speakers**
![](https://assets.underline.io/uploads/markdown_image/1/image/8ce6235e957d31cb5e6f9097cc6f6972.jpg)
Jessica Li, MD/PhD Candidate
![](https://assets.underline.io/uploads/markdown_image/1/image/9950ef2e0237f54cb3d8280334d42491.jpg)
Charles Lopresto, DO

### Sponsor
AMA would like to thank Laurel Road for sponsoring a $10,000 grand prize for the winner of the AMA Research Challenge. Laurel Road is not involved in the judging or scoring process to select the winner. The winner will be announced on Dec. 7 during the AMA Research Challenge finals. Payment of the grand prize will be directly from Laurel Road. See the AMA Research Challenge Official Rules for terms and conditions.

![](https://assets.underline.io/uploads/markdown_image/1/image/611e331d4a0eba0920f240cfc04b4d77.png)

**There is no registration fee for this event! **

Register to explore research in a variety of topics. AMA members get exclusive access to score posters in the semifinals.

**Please use the link below to register for free if you have not already done so:**

https://www.ama-assn.org/about/events/ama-research-challenge

Registration Is Required

AMA Research Challenge 2022

Welcome to the largest national, multi-specialty research event featuring research from 750 medical students, residents and fellows, and international medical graduates.


Background
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs) are a group of autoimmune disorders that result in systemic inflammation with distinct clinical manifestations. These further categorize this pathology into three unique subtypes. First, Granulomatosis with Polyangiitis (GPA) (previously Wegener's Granulomatosis) is associated with the PR3–ANCA autoantibody and is known for its inflammatory destruction of upper and lower respiratory tracts, as well as renal small vessels. Second, Microscopic Polyangiitis (MPA) is another type of AAV that may present similarly to GPA; however, distinct features that separate this subtype include the absence of upper respiratory involvement and the MPO–ANCA antibody predominance. Finally, Eosinophilic Granulomatosis with Polyangiitis (EGPA) (previously Churg-Strauss syndrome) is also associated with MPO-ANCA. However, this rare type of AAV presents with asthma and eosinophilia.  The pathogenesis of all three types of AAVs includes destructive inflammation that begins intravascularly and progresses to tissue destruction and end-organ damage. Early management strategies typically involve glucocorticosteroids, and rituximab and cyclophosphamide are frequently included in patients with severe disease. 

Case Presentation
Here, we present a clinical case of MPO–ANCA positive vasculitis with a benign presentation of epigastric pain and poor appetite that rapidly progresses into systemic inflammation with pulmonary and renal manifestations. In this case, an 81-year-old female with no known medical history presents with mild chronic epigastric pain and poor appetite. Clinically, the patient was stable and well-appearing, with no apparent signs of deterioration at the bedside. Early laboratory investigations were consistent with mild anemia and chronic kidney disease. Lung imaging demonstrated diffuse airspace opacities and tree-in-bud pulmonary nodules.  Screening for infectious etiologies, including bacterial (including tuberculosis), viral, and fungal, were negative. As inflammatory markers continued to uptrend, secondary inflammatory etiologies were explored, resulting in the discovery of a positive MPO–ANCA autoantibody test.  This was followed by rapid deterioration into acute hypoxemic respiratory failure, requiring mechanical ventilation, and complicated by diffuse alveolar hemorrhage, in addition to severe anemia requiring multiple transfusions and acute kidney failure.

Discussion
This clinical case highlights the rapidly progressing nature of AAVs and the frequent benign presentations that can masquerade as this aggressive pathology. As the literature continues to evolve and offer recommendations on the management of these rare autoimmune diseases, early recognition, diagnosis, and intervention are essential to minimize mortality and morbidity rates and offer patients a relapse-free prognosis and improved clinical outcomes.


poster

Welcome to the **largest national, multi-specialty research** event featuring research from more than 750 medical students, residents, fellows, and international medical graduates.

Use the buttons below or navigation menu to access the event.
 

[![](https://assets.underline.io/markdown_image/1/image/20e1e77aa954efeac7a15d84c85eb383.jpg)](https://underline.io/events/468/sessions?searchGroup=all)
 
[![](https://assets.underline.io/markdown_image/1/image/068522d81aca0b68a369787c7dfccd1b.jpg)](https://underline.io/events/468/posters?searchGroup=all)
 
[![](https://assets.underline.io/markdown_image/1/image/d3971ece79d5e5575b178bdc2c8bbfae.jpg)](https://underline.io/events/468/help-desk)
 

#### Event Details

The 2024 American Medical Association Research Challenge includes a poster symposium hall and a semifinals hall with the top scored research. **Log in at any time throughout the event to:**

* **View posters and presentations** on a variety of topics and specialties
* **Score posters in the semifinals** to help decide the top five finalists (AMA member exclusive opportunity)
* **Connect with participants** and provide feedback to help advance their research

At the conclusion of this event, the AMA will announce the top five finalists who will advance to the AMA Research Challenge finals, present their research to an elite panel of judges, and compete to win a $10,000 grand prize presented by Laurel Road. 

### Grand Prize Sponsor

AMA would like to thank Laurel Road for sponsoring a $10,000 grand prize for the winner of the AMA Research Challenge. Laurel Road is not involved in winner selection process. The winner will be announced on February 20, 2025 during the AMA Research Challenge finals. Payment of the grand prize will be directly from Laurel Road. See the AMA Research Challenge Official Rules for terms and conditions.

[![](https://assets.underline.io/uploads/markdown_image/1/image/611e331d4a0eba0920f240cfc04b4d77.png)](https://www.laurelroad.com/)

You need to log in with the email address you registered with. Access credentials have been sent to your email. 

Please be sure to check your spam and other email folders if you do not see an email confirmation right away.

Please log in to explore this event.

**There is no registration fee for this event! **

If you have used the AMA Research Challenge platform previously, please click the ‘Log-in’ button below. If you do not remember your password, please click “forgot password” and follow prompts to reset your password. After resetting your password, please refresh your webpage to access the platform.

Register to explore research in a variety of topics. AMA members get exclusive access to score posters in the semifinals. 

**Please use this link [here](https://www.ama-assn.org/member-benefits/events/ama-research-challenge) to register for free.**

Join the AMA Research Challenge poster symposium and semifinals to explore top research posters and score posters in the semifinals (an AMA member exclusive opportunity).

Background
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) encompasses autoimmune disorders marked by inflammation and damage to small- and medium-sized blood vessels, alongside the presence of circulating ANCA. Prompt diagnosis and treatment are critical for patient survival, necessitating interventions like hemodynamic support, high-dose corticosteroids, immunosuppressants, and plasmapheresis (PLEX).
Case Presentation
We report a case of a 65-year-old female with a history of hypertension and Chronic Kidney Disease (CKD) Stage 4, secondary to rapidly progressive glomerulonephritis caused by Microscopic Polyangiitis. She presented to the emergency department with a productive cough, subjective fever, chills, body aches, and generalized weakness. Her initial vitals were stable. Laboratory results indicated normocytic anemia, leukocytosis, and acute kidney injury on CKD. Chest X-ray revealed left multifocal pneumonia. She was treated with intravenous Ceftriaxone and Azithromycin and transfused with one unit of packed red blood cells (PRBC). On the following day, she developed shortness of breath and acute hypoxic respiratory failure, necessitating 6L nasal cannula oxygen. Persistent symptomatic anemia without overt bleeding led to the suspicion of diffuse alveolar hemorrhage (DAH) amidst her rapid progressive glomerulonephritis. A CT scan of the chest, abdomen, and pelvis disclosed extensive multilobar airspace disease. Methylprednisolone 1g IV daily was initiated, followed by prophylactic intubation. Bronchoscopy confirmed DAH. The patient was started on Rituximab and underwent five sessions of PLEX. She was subsequently weaned off mechanical ventilation and discharged to a subacute rehabilitation facility, with instructions to follow up with Nephrology, Rheumatology, and her primary care physician.
Discussion
The triad of dyspnea, anemia, and lung infiltrates was pivotal in raising suspicion of DAH in this patient. Previous studies have noted a correlation between age at presentation and mortality risk in AAV patients with DAH. Hemoptysis is not always present and may be absent in up to 33% of cases. Prompt and aggressive treatment is essential to prevent complications in ANCA vasculitis patients. The mainstays of remission induction therapy in AAV include corticosteroids and cyclophosphamide or rituximab. PLEX serves as an adjunct to immunosuppressive therapy, effectively removing circulating pathogenic antibodies that contribute to vascular damage.

An Atypical Presentation of Diffuse Alveolar Hemorrhage in Microscopic Polyangiitis

Background
Hydroxychloroquine is a medication that is used as an antimalarial or a disease-modifying anti-rheumatic drug. It has rare side effects like cardiomyopathy, hematological abnormalities, and dermatological manifestations. Hydroxychloroquine-induced hepatotoxicity is a rare yet serious adverse effect, requiring further investigation and should be considered a differential diagnosis in patients with connective tissue diseases and acute liver issues.

Case Presentation
This is a 31-year-old female with a history of uveitis and systemic lupus erythematous (SLE), which was diagnosed two months prior to presentation with a positive anti-dsDNA and anti-Smith antibodies after having initial findings of uveitis, joint pain, and hair loss. She was started on Hydroxychloroquine 200 mg twice daily a month after being diagnosed. A month after Hydroxychloroquine initiation, she developed abdominal pain and vomiting. Subsequently, she took two doses of acetaminophen, dextromethorphan, and phenylephrine, one in the morning and one in the evening for one day at the recommended dosage. The following day, at urgent care, she was diagnosed with viral gastritis and received one gram of acetaminophen. Three days later, in the emergency room, she had acute liver failure with peak aspartate aminotransferase – 4731 U/L and alanine aminotransferase – 5109 U/L. She endorsed minimal social alcohol use, and tested negative for Human Immunodeficiency Virus, Cytomegalovirus, Hepatitis A, B, and C. Liver biopsy showed portal tracts with mild-moderate expansion by mixed inflammatory infiltrates including scattered eosinophils with mild interface activity focally close to bridging inflammation – findings consistent with drug-induced liver injury. Hydroxychloroquine was held, and she was admitted for conservative management. Repeat liver function tests showed down trending during the patient’s hospitalization – normalizing two months post-discharge.

Discussion
Hepatotoxicity due to hydroxychloroquine use is rare but has been reported in eight other cases. The mean age of onset was 31.6 years old, mean cumulative dose used was 342 mg daily, and mean duration of hydroxychloroquine use was 53.8 days. Two patients expired, while the others recovered after discontinuing hydroxychloroquine. The mechanism of hydroxychloroquine-induced hepatotoxicity is unclear but may be related to its metabolism by cytochrome P450 in the liver. Hydroxychloroquine may share a similar pathophysiologic mechanism of toxicity as chloroquine due to their similar molecular structure. A study showed that rats treated with both bacterial lipopolysaccharide and chloroquine developed signs of liver injury, suggesting that the quinolone class of drugs can induce liver injury in those with concurrent inflammatory conditions. Early detection and termination of hydroxychloroquine is crucial in preventing progression to fulminant hepatic failure.

From Rheumatology to Hepatology: A Patient’s Unexpected Hydroxychloroquine Journey

Background
Ferritin levels serve as an essential marker for iron status and aid in diagnosing iron deficiency anemia. However, ferritin is also an acute-phase reactant and is often elevated in inflammatory conditions. Hyperferritinemia refers to markedly elevated ferritin levels over 10,000 μg/L and is associated with various disorders, including multisystem inflammatory syndrome in children (MIS-C), macrophage activation syndrome (MAS), adult-onset Still’s disease (AOSD), and hemophagocytic lymphohistiocytosis (HLH). These disorders are often difficult to diagnose, leading to life-threatening outcomes. There is growing interest in investigating ferritin as a biomarker for diseases. Ferritin is commonly included in patients’ initial workups, yet its results are underutilized due to seemingly complex clinical interpretations.
Case Presentation
Here, we present two case reports illustrating the utilization of ferritin in two rare disorders: AOSD and HLH. The first case follows a 37-year-old male presenting with a pruritic rash, flu-like symptoms, joint pain, fever, and chills. After multiple emergency department (ED) visits, the patient was found to have hyperferritinemia to 88,000 μg/L and met the Yamaguchi criteria for AOSD. He was treated with pulse- dose steroids, resulting in a swift resolution of symptoms. The second case follows a 50-year-old female presenting with sepsis secondary to recurrent axillary skin infections. Her complicated hospital course prompted her transfer to the intensive care unit (ICU), and her labs were significant for hyperferritinemia to 39,671 μg/L, which was essential to differentiate between rheumatological and hematological etiologies. She was diagnosed with diffuse large B-cell lymphoma and, unfortunately, passed away.
Discussion
Recognizing the role of hyperferritinemia is crucial in assessing mortality risk in these diseases. The etiologies of marked hyperferritinemia are complex and often unclear, necessitating consideration of HLH and MAS in critically ill patients with elevated ferritin levels. Hematological malignancies, which may trigger HLH, highlight the need to differentiate these life-threatening conditions. Further research is needed to aid in the effective diagnosis and management of hyperferritinemia-associated disorders, as prompt diagnoses are critical in improving patient outcomes.

Mining for a diagnosis: rare causes of hyperferritinemia

Background
Axial gout is an atypical presentation characterized by the deposition of monosodium urate crystals in the spine. Unlike peripheral joints, spinal gout can pose diagnostic challenges due to its nonspecific presentation and the inability to obtain joint fluid for crystal analysis. This case highlights severe subacute back pain where dual-energy computed tomography (DECT) was crucial in diagnosing spinal gout.

Case Presentation
A 73-year-old male with a history of gout, chronic kidney disease, hypertension, and hyperlipidemia presented with worsening subacute back pain. Initial MRI imaging revealed phlegmonous changes with elevated inflammatory markers. IR-guided drainage was performed, and the cultures were negative. He was treated with analgesics, resulting in symptom improvement. Two weeks later, he experienced worsening back pain, limiting his ambulation to using a wheelchair, and even the slightest movement exacerbated the pain.
Repeat MRI imaging revealed features concerning osteomyelitis/discitis. Given the negative cultures, episodic nature of the disease, symptom improvement with NSAIDs, and elevated uric acid on admission, there was a high suspicion for gout and low suspicion for infection. A DECT scan of the lumbar spine was performed, showing monosodium urate crystal deposition with erosive arthritis consistent with lumbar spinal gout. The patient was started on allopurinol and colchicine, resulting in only mild improvement in back pain. As cultures were negative, infection was ruled out. He was then started on prednisone, resulting in significant pain relief.

Discussion
Spinal gout can be confused with pathologies like vertebral osteomyelitis, malignancy, epidural abscess, and spondylarthritis. Features such as elevated inflammatory markers, fever, and leukocytosis are common to both infection and gout. MRI and CT can show phlegmonous changes and erosions, but these findings can be non-specific. DECT, using two energy levels of X-ray beams (80 kV and 140 kV), differentiates structures based on their attenuation characteristics. After post-processing, materials are color-coded for identification, with urate often coded green. DECT’s sensitivity and specificity for gout are 90% and 83%, respectively, and it can diagnose gout even with negative synovial fluid analysis. An accurate diagnosis with DECT helps avoid unnecessary interventions like biopsy and drainage. Early and accurate detection is crucial for preventing severe complications, including neurological deficits from the compressive effects of tophi. Sudden onset back pain, elevated uric acid, and improvement with NSAIDs should prompt consideration of spinal gout. Aggressive uric acid control is needed to avoid recurrence, with a target level of <6 mg/dL.

References:
1. Harlianto NI, Harlianto ZN. Patient characteristics, surgical treatment, and outcomes in spinal gout: a systematic review of 315 cases. European Spine Journal. 2023 Nov;32(11):3697-703.
2. Lumezanu E, Konatalapalli R, Weinstein A. Axial (spinal) gout. Current rheumatology reports. 2012 Apr;14:161-4.
3. Stauder SK, Peloso PM. Dual-Energy computed tomography has additional prognostic value over clinical measures in gout including tophi: A systematic literature review. The Journal of Rheumatology. 2022 Nov 1;49(11):1256-68.
4. Gamala M, Jacobs JW, Van Laar JM. The diagnostic performance of dual energy CT for diagnosing gout: a systematic literature review and meta-analysis. Rheumatology. 2019 Dec 1;58(12):2117-21.

Pain in the Spine - A Crystalline Narrative

Intro
Mix connective tissue disease (MCTD) is a rare autoimmune disease consisting of at least two of the following connective tissue diseases: systemic lupus, systemic sclerosis, polymyositis, dermatomyositis, and rheumatoid arthritis. Anti-U1-ribonucleoprotein is present.
Case
A 45-year-old female with PMH of MCTD presents to the hospital after routine lab work showed low hemoglobin. The patient went to urgent care for a cough; labs showed low hemoglobin, and the patient was recommended to go to ED. She had an MCTD flare three months ago and followed up with a rheumatologist. Since her flare-up a few months ago, she has been taking both methotrexate and dapsone, along with a prednisone taper, currently taking prednisone 10mg daily. She reported a 20-pound weight loss over the past three months due to poor appetite during a recent flare. She denies any history of fevers, chills, chest pain, shortness of breath, abdominal pain, constipation, diarrhea, numbness, tingling, or joint pain. The patient denies any history of epistaxis, hematemesis, melena, hematochezia, menorrhagia, or recent traumas. 
Results 
The patient was admitted for a blood transfusion and workup for anemia. Rheumatology and hematology/oncology were consulted for MCTD and anemia workup. During her hospital stay, the patient's lab work demonstrated the development of several autoimmune conditions along with her original diagnosis of MCTD, which led to pulmonology and nephrology being consulted. Based on labs and imaging, anemia of chronic disease, autoimmune hepatitis, lupus nephritis, and pleural effusion were found, requiring extensive outpatient follow-up with Rheumatology, Pulmonology, Hematology/Oncology, Nephrology, and her Primary Care physician. 
Conclusion/discussion 
Mixed connective tissue disease can be a curable condition but can also have a life-threatening presentation. The prognosis depends on which organs are affected, with pulmonary hypertension being the most common cause of death. The mortality rate varies between 8%-36%. Patient education is vital in controlling this disease and managing someone’s quality of life. MCTD needs to be managed by an interprofessional team to address each organ being affected and have regular follow-ups. Without regular follow-up, MCTD patients can have disease progression and further complications. In the case presented, the patient requires four referrals to specialists and a follow-up with her PCP.


The Importance of Longterm Management of Mixed
Connective Tissue Disease: A Case Report

Introduction:
Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are rapidly developing mucocutaneous reactions, existing as a spectrum based on skin involvement, generally triggered by medications. SJS/TEN-like systemic lupus erythematosus (SLE) is described in
several case reports, although it remains uncommon. It presents as a diagnostic conundrum given that this bullous rash is not typically seen with lupus. Here, we describe a case of SLE masquerading as SJS.

Case Presentation:
A 25-year-old male with mixed connective tissue disease (MCTD) and
attention-deficit/hyperactivity disorder presented with a diffuse rash and oral mucosal involvement after a sore throat and fever. He had recently started Modafinil. Upon examination, he appeared ill, with a generalized maculopapular rash. Lab-work revealed thrombocytopenia, normocytic anemia, acute kidney injury (AKI), and transaminitis. Rheumatology was consulted, and initially recommended monitoring off Modafinil, with further
work-up, including a skin biopsy (as rash appeared atypical for that seen in MCTD or lupus). Other consultants were also involved, including Dermatology, Nephrology, and Hematology-Oncology. Patient underwent a kidney biopsy and was started on IV Methylprednisolone 150 mg for three days, followed by Prednisone.
Interestingly, skin biopsy findings indicated features of SJS (separation of the necrotic epidermis from the dermis, perivascular lymphocytic infiltrate); however, there were also atypical features (mixed-cell infiltrate with neutrophils). The sample was then sent out for a second opinion. The patient developed respiratory failure and pulmonary edema, necessitating intubation and transfer to the Intensive Care Unit for pressor support due to cardiogenic shock [echocardiogram showed an ejection fraction of less than 30%]. Given clinical deterioration with
worsening cytopenias, transaminitis, AKI, hypocomplementemia, positive antinuclear-antibody, and double-stranded DNA (dsDNA), he was switched to IV Methylprednisolone 1 gram for three days, followed by a steroid taper. Elevated triglycerides (765 mg/dl) and ferritin (15,084 ng/ml) raised concerns for HLH, leading to the initiation of Rituximab (literature has shown use off-label
for SLE, and benefit in certain viral-induced HLH). Kidney biopsy revealed acute tubular necrosis and Class 1 lupus nephritis. Ultimately, direct
immunofluorescence of skin biopsy showed granular deposition of immunoglobulins and complement consistent with SLE.

The patient was also started on continuous renal replacement therapy. After receiving intravenous immunoglobulin (IVIG) for four days and Cefepime for Escherichia coli and Klebsiella bacteremia, he was weaned off pressors. Improvements were seen in complements, dsDNA, C-reactive protein, ferritin, and cytopenias. By discharge, dialysis was no longer needed, and he was started on Hydroxychloroquine, Prednisone taper, Atovaquone, and Spironolactone.

Discussion:
This case highlights the diagnostic and therapeutic challenges of managing lupus-associated SJS/TEN. Initial biopsy findings were consistent with SJS, but atypical cells and subsequent immunofluorescence confirmed SLE. Key clinical indicators—hypocomplementemia, elevated dsDNA, and steroid-responsive cytopenias—pointed to an SJS/TEN-like lupus. Management was further complicated by conflicting reports on the use of steroids in SJS/TEN and the development of HLH, requiring an aggressive treatment approach. Differentiating SJS/TEN-like SLE from drug-induced SJS/TEN is crucial for appropriate management and
prognosis.

Unmasking the Rash: Unveiling the Mystery Behind a Curious Skin Reaction

Background
Subclavian artery stenosis can lead to retrograde flow from an ipsilateral vertebral
artery which can result in significant cerebrovascular symptoms (i.e. Vertebral Basilar
Insufficiency syndrome, VBI). An Aberrant Right Subclavian Artery (ARSA) is an anatomical
variation resulting in the right subclavian artery arising from the aortic arch rather than the right
brachiocephalic artery. Our patient presented with both conditions, each exceedingly rare.
Case Presentation
We present the case of a 76-year-old female with a past medical history significant for bilateral rheumatoid arthritis of the hands, hypothyroidism, and osteoporosis who presented to the emergency department for intermittent dizziness, near syncope, and lightheadedness for 2 days. Notably, the patient reported 3 episodes of incomprehensible speech. Physical exam demonstrated absent radial and ulnar pulses on the right with a systolic blood pressure difference from left to right arm of >60 mmHg. Diagnostic imaging confirmed a completely occluded and calcified ARSA. After a careful multidisciplinary discussion, the diagnosis of an occluded ARSA causing VBI was made. A right subclavian-carotid transposition procedure was performed to restore adequate blood flow and alleviate her symptoms. Her postoperative course was complicated by Horner syndrome.

Discussion
While ARSA is often asymptomatic, this patient exhibited symptoms due to the occlusion. Those that are symptomatic most commonly present with dysphagia, dyspnea, and coughing; VBI being exceedingly rare. Since the patient’s right subclavian artery was completely occluded at its origin and proximal segments, the right upper extremity received its blood flow through retrograde flow from the ipsilateral vertebral artery, which resulted in decreased cerebral perfusion without ischemic changes. While endovascular treatment can be considered first line in treating subclavian artery stenosis, the option of a carotid-subclavian bypass or carotid-subclavian transposition are available. Considering the anatomic variation present on this patient, the completion of a subclavian-to-carotid transposition was performed with immediate correction of blood flow. This case highlights the importance of recognizing rare vascular anomalies and unique pathophysiological presentations and the need for tailored surgical approaches in complex presentations.

A Case of Vertebral Basilar Insufficiency in the Setting of a Completely Occluded Aberrant Right Subclavian Artery.

Introduction/Objective
The sinus of Valsalva is the most proximal section of the ascending aorta near the aortic root. Sinus of Valsalva aneurysms (SOVA) is a rare congenital or acquired condition of the aortic root. Ruptured SOVA typically involves another chamber of the heart. Free wall rupture of the SOVA is normally associated with sudden death due to tamponade. We report a case of blunt trauma resulting in the rupture of the non-coronary sinuses of Valsalva, which was successfully repaired.

Case Presentation
The patient is a 47-year-old male with a past medical history of left thoracotomy and sternotomy for penetrating trauma who recently sustained blunt chest trauma at work. The patient was transferred from an outside hospital due to concern for thoracic aortic injury with active extravasation into the pericardium. Upon arrival, the patient complained of a brief loss of consciousness, chest pain, and shortness of breath. On the primary survey, the patient required BiPAP and esmolol drip. 
Transthoracic echocardiogram (figure 1A) and chest CTA (figure 1B) revealed a rupture of the sinus of Valsalva.
Figure 1A: The transthoracic echocardiograph reveals a rupture of the sinus of Valsalva. The rupture is near the aortic root's non-coronary cusp (NCC). Also visible are the right ventricle (RV), left ventricular outflow tract (LVOT), right coronary cusp (RCC), and ascending aorta.
Figure 1B: Axial view of chest CT showing disruption of the non-coronary cusp (NCC) with contained posterior hematoma within the mediastinum. The left coronary cusp (LCC) and right coronary cusp (RCC) are visualized.
The patient underwent a redo sternotomy, revealing a rupture of the left and non-coronary aortic root sinuses. He received a biological aortic root and valve replacement. After a prolonged hospital course, he was discharged home and resumed all normal activities upon outpatient follow-up.

Discussion
A literature search did not identify any reports of long-term survival following emergency surgical intervention for traumatic, free rupture of the Sinuses of Valsalva. We identified reports of non-traumatic ruptures of the SOVA into the pericardium causing tamponade. The unique feature of this case was the acute traumatic cause of the rupture, the distinct CT and TTE findings, and the successful aortic valve replacement. The history of sternotomy for penetrating trauma possibly contributed to the patient’s survival.

Conclusion
Traumatic free wall ruptured sinus of Valsalva into the mediastinum can show specific radiological findings; rapid surgical correction should ensue.

Successful Repair of a Ruptured Sinus of Valsalva Following Blunt Chest Traumatic Injury

Background
Primary male breast cancer with a synchronous second primary neoplasm is rare. Prostate-specific membrane antigen (PSMA) PET scan uses a tracer for detection of metastatic prostate cancer. Research has shown PSMA is highly expressed in triple negative breast cancer. Here, we report a case of a 75-year-old male with primary prostate cancer and synchronous primary breast cancer which was discovered on prostate-specific membrane antigen (PSMA) scan. To our knowledge, this is the first case showing primary prostate and primary breast cancer in a male with osseous metastasis identified on PSMA scan. 

Case Presentation
Patient is a 75-year-old male presenting with PSA of 180. Transrectal ultrasound guided prostate biopsy was positive for Gleason grade group 4 (4+4= 8) prostate cancer in 11 out of 12 cores with extra-prostatic extension. PSMA scan was obtained, showing increased PSMA expression consistent with widespread nodal and osseous metastatic prostate cancer. In addition, there was increased radiotracer uptake within the left breast soft tissue and enlarged left axillary lymph node, which may represent primary breast neoplasm versus metastatic prostate cancer. 

A palpable left breast mass in the breast underwent biopsy. Pathology was consistent with invasive ductal carcinoma, grade 1, measuring 11mm in the greater linear extent, ER positive, PR positive, HER2 negative. 

Prostate specific acid phosphate (PSAP) and NKX3.1 staining was negative, ruling out metastatic prostate cancer in the breast. 

The patient underwent left breast radical mastectomy with axillary contents. Pathology was consistent with invasive moderately differentiated ductal carcinoma 3.6cm (grade 2), adjacent to and arising from encapsulated papillary carcinoma. Margins negative for malignancy. One of fourteen axillary nodes positive for metastasis. Staged as pT2pN1a. Tamoxifen 20mg daily for 5 years and Lupron 45mg q6 months was initiated. PSA will be rechecked q6 months.


Discussion
This case is an example of a PSMA scan ordered for evaluation of prostate cancer metastasis incidentally found a primary breast cancer. It is an important case as providers should be aware that uptake in PSMA scans have the potential to be related to a second primary cancer.

Detection of primary male breast cancer with PSMA PET scan

A non-common cause of constipation in an 86-year-old female with abdominal pain.
EstrellaAcosta, Katty MD1, and RodriguezMichel, Francisco J.MD2 
1Rotatory Internship Program, 2,3 Internal Medicine Department. Hospital Buen Samaritano, Aguadilla, PR.

Introduction: A cholecystocolonic fistula is an abnormal connection between the gallbladder and the colon. This condition is rare but can lead to significant clinical issues in patients presenting with cholelithiasis.
Case Summary: We present an 86-year-old woman admitted to our institution with the diagnosis of intestinal obstruction. The computed-tomography examination of the abdomen was consistent with a gallstone at the sigmoid colon. Intraoperatively, the calculus necrotized the gallbladder wall and formed a cholecystocolonic fistula, presented with severe colonic inflammation requiring a segmental resection of the colon with end-to-end anastomosis and cholecystectomy with a traditional open approach. The abdomen was closed. Her post-operative course was complicated by anuria that did not respond to aggressive intravenous fluid, persistent respiratory failure and asystole that did not respond to ACLS protocol.
Discussion: Inflammatory process for a long period of time allows the entrance of gallstone into the bowel through a fistulous tract. This case illustrates that a high index of suspicious is necessary in patients with cholelithiasis history, constipation, and radiologic evidence of pneumobilia.
Conclusion: Timely diagnosis and appropriate surgical management are crucial for favorable outcomes.



Premium content

Next from AMA Research Challenge 2024

An Atypical Presentation of Diffuse Alveolar Hemorrhage in Microscopic Polyangiitis

Similar lecture

Case Report: Patient with an ANCA negative and treatment-resistant Granulomatosis Polyangitis with CNS involvement.