United States

Introduction: 
Tracheobronchopathia osteochondroplastica (TO) is a rare idiopathic benign disease of the large airways with a clinical prevalence of 0.2% to 0.7%. It is characterized by osteocartilaginous nodules that arise from the anterolateral aspects of the airway walls and project into the lumen of the trachea and bronchi. While typically asymptomatic, patients may experience dyspnea, excessive mucus production, or hemoptysis. The gold standard for diagnosis is a computed tomography (CT) scan and bronchoscopy, which often discover TO secondary to another condition. While no specific treatments exist for TO, the prognosis is generally favorable. This case report discusses a presentation of a male whose potential TO was incidentally discovered while evaluating his pleural effusion. 

Case Presentation:
A 70-year-old nonsmoker male with diabetes mellitus, end-stage renal disease on hemodialysis, and atrial fibrillation (not on anticoagulation) presented for continued evaluation of his unexplained bilateral pleural effusion.

The patient underwent a thoracentesis that confirmed an exudative pleural effusion (protein = 3.5 g/dl and LDH &gt; 2/3 upper limit of normal). A follow-up CT scan of the chest revealed that the pleural effusion was more prominent on the left side, bibasilar atelectasis, compressive and rounded left lower lobe (LLL) atelectasis, scattered calcified granulomas, and left-sided pulmonary infiltrates.

To further evaluate, a bronchoscopy with bronchoalveolar lavage (BAL) and transbronchial biopsy was conducted the following month. Although the BAL and transbronchial biopsy results were negative, the bronchoscopy revealed yellowish tracheal nodules and bronchial nodules in the right mainstem and left upper lobe bronchi, suggesting potential Tracheobronchopathia Osteochondroplastica.

Discussion:
TO is often a difficult condition to diagnose due to its benign nature and unknown etiology. This patient’s bronchoscopy revealing yellowish nodules along his tracheal and bronchial walls, CT showing potential calcifications, and biopsy indicating no malignancies collectively suggest a possible presentation of TO. There are no established follow-up guidelines, and it may be useful to see if the nodules increase in size or number over time and how this progression could affect the patient&#39;s symptoms and long-term outcomes. More work is needed to see if TOs are associated with or increase the risk for lung pathologies, such as pleural effusions, among other systemic diseases.

Conclusion:
As TOs have very few effects, a diagnosis of TO is often made incidentally when evaluating other primary conditions. This patient’s presentation suggestive of tracheobronchopathia osteochondroplastica contributes to the limited literature on how TO may present.


References:
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5474380/
https://www.mayoclinicproceedings.org/article/S0025-6196(19)30101-6/fulltext

AMA Research Challenge 2024 

Unveiling Hidden Nodules: An Incidental Discovery of Tracheobronchopathia Osteochondroplastica

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AMA Research Challenge 2023

Join the AMA Research Challenge poster symposium and semifinals to explore top research posters, score posters in the semifinals (an AMA member exclusive opportunity) and attend education sessions with experienced physicians to learn tips to advance your research and career. 

Introduction: 
Tracheobronchopathia osteochondroplastica (TO) is a rare idiopathic benign disease of the large airways with a clinical prevalence of 0.2% to 0.7%. It is characterized by osteocartilaginous nodules that arise from the anterolateral aspects of the airway walls and project into the lumen of the trachea and bronchi. While typically asymptomatic, patients may experience dyspnea, excessive mucus production, or hemoptysis. The gold standard for diagnosis is a computed tomography (CT) scan and bronchoscopy, which often discover TO secondary to another condition. While no specific treatments exist for TO, the prognosis is generally favorable. This case report discusses a presentation of a male whose potential TO was incidentally discovered while evaluating his pleural effusion. 

Case Presentation:
A 70-year-old nonsmoker male with diabetes mellitus, end-stage renal disease on hemodialysis, and atrial fibrillation (not on anticoagulation) presented for continued evaluation of his unexplained bilateral pleural effusion.

The patient underwent a thoracentesis that confirmed an exudative pleural effusion (protein = 3.5 g/dl and LDH > 2/3 upper limit of normal). A follow-up CT scan of the chest revealed that the pleural effusion was more prominent on the left side, bibasilar atelectasis, compressive and rounded left lower lobe (LLL) atelectasis, scattered calcified granulomas, and left-sided pulmonary infiltrates.

To further evaluate, a bronchoscopy with bronchoalveolar lavage (BAL) and transbronchial biopsy was conducted the following month. Although the BAL and transbronchial biopsy results were negative, the bronchoscopy revealed yellowish tracheal nodules and bronchial nodules in the right mainstem and left upper lobe bronchi, suggesting potential Tracheobronchopathia Osteochondroplastica.

Discussion:
TO is often a difficult condition to diagnose due to its benign nature and unknown etiology. This patient’s bronchoscopy revealing yellowish nodules along his tracheal and bronchial walls, CT showing potential calcifications, and biopsy indicating no malignancies collectively suggest a possible presentation of TO. There are no established follow-up guidelines, and it may be useful to see if the nodules increase in size or number over time and how this progression could affect the patient's symptoms and long-term outcomes. More work is needed to see if TOs are associated with or increase the risk for lung pathologies, such as pleural effusions, among other systemic diseases.

Conclusion:
As TOs have very few effects, a diagnosis of TO is often made incidentally when evaluating other primary conditions. This patient’s presentation suggestive of tracheobronchopathia osteochondroplastica contributes to the limited literature on how TO may present.


References:
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5474380/
https://www.mayoclinicproceedings.org/article/S0025-6196(19)30101-6/fulltext

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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.

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Title: Breaking Barriers in Amniotic Fluid Embolism Management with Interventional Radiology

Introduction: 
Amniotic fluid embolism (AFE) is a rare obstetric emergency with high mortality rates. Prompt recognition and supportive, multidisciplinary treatment is vital for enhancing patient outcomes. This case highlights the successful treatment of AFE with interventional radiology (IR). 

Background: 
Amniotic fluid embolism is a rare medical emergency that can occur during labor and postpartum. The pathogenesis is theorized to be due to a release of inflammatory cytokines reacting to amniotic fluid in the maternal bloodstream, causing cardiovascular collapse, respiratory failure, and disseminated intravascular coagulation (DIC) [1]. Diagnosis is based on clinical findings of hypoxia, hypotension, and coagulopathy in peripartum or postpartum women [2].

Treatment is supportive, including respiratory, cardiac, hemostatic, and hemodynamic resuscitation. This requires a multidisciplinary team to be heavily involved in the patient’s care, with primary contributions from obstetrics, critical care, cardiology, and pulmonology [3]. 

The prognosis is grim. Studies suggest maternal mortality ranges from 20% to 60% [4]. With placenta accreta spectrum disorder, mortality doubles [5]. 

Case: 
Our patient is a 27-year-old now G2P2 female with a history of placenta accreta who developed seizure-like activity, hypotension, and respiratory distress shortly after delivery, all concerning for AFE. Initial labs revealed a fibrinogen of 69 and INR of 1.2, findings consistent with disseminated intravascular coagulation (DIC). She developed pulseless ventricular tachycardia that spontaneously resolved and was transferred to our facility for further workup.

Upon arrival, she was cyanotic, tachypneic, tachycardic, and hypotensive. CXR revealed pulmonary edema. Bedside echocardiogram revealed an ejection fraction of 35-40%. Troponins were elevated at 50,000. MTP was subsequently activated. 

Plans were made to undergo CTA C/A/P to exclude a pulmonary embolism; however, due to hemodynamic instability, she was taken to the IR suite for embolization of the bilateral uterine and internal iliac arteries. Following the procedure, her vital signs normalized without vasopressor support. 

Later that evening, she became hypotensive and tachycardic. CXR was concerning for flash pulmonary edema. She developed two episodes of supraventricular tachycardia that responded to vagal maneuvers. Intubation was performed for hypoxemia and respiratory distress. 

After a multidisciplinary discussion, the decision was made to take the patient for a repeat diagnostic pelvic angiogram, which revealed brisk arterial extravasation from the left inferior epigastric artery which was not conspicuous on the initial angiogram. IR then performed coil embolization of the left inferior epigastric artery. A conventional pulmonary arteriogram was also performed to exclude a large pulmonary embolus given her worsening hypoxemia. 

She was subsequently stabilized and remained so for the duration of her admission, receiving a total of 14 pRBCs, 13 FFP, 3 platelets, and 3 cryoprecipitates.

Discussion:
IR’s prompt identification and treatment of arterial extravasation were monumental in stabilizing the patient and mitigating the risk of further deterioration. In obstetric emergencies, especially those involving severe hemorrhage, rapid and precise interventions are crucial. IR offers minimally invasive techniques that can effectively and quickly control emergent bleeding and provide patient stabilization without the need for vasopressor support or general anesthesia.

There is currently no specific research or case documentation highlighting the significance of IR procedures in managing AFE. Further studies are warranted to analyze the significance of incorporating IR into the standard management of AFE across medical facilities. 

While interventional radiology may not traditionally be a primary component of the management for AFE, its inclusion in this case demonstrates its ability to significantly optimize patient outcomes and warrants more frequent consideration in similar emergencies.

Conclusion: 
The prompt integration of IR into our multidisciplinary approach played a pivotal role in the successful management of AFE, highlighting the significant impact IR can have on optimizing patient outcomes. 

References:
Cavoretto PI, Rovere-Querini P, Candiani M. Toward Risk Assessment for Amniotic Fluid Embolisms. JAMA Netw Open. 2022;5(11):e2242850. Published 2022 Nov 1. doi:10.1001/jamanetworkopen.2022.42850
Sundin CS, Mazac LB. Amniotic Fluid Embolism. MCN Am J Matern Child Nurs. 2017;42(1):29-35. doi:10.1097/NMC.0000000000000292 
Pacheco LD, Clark SL, Klassen M, Hankins GDV. Amniotic fluid embolism: principles of early clinical management. Am J Obstet Gynecol. 2020;222(1):48-52. doi:10.1016/j.ajog.2019.07.036
Clark SL. Amniotic fluid embolism. Obstet Gynecol. 2014;123(2 Pt 1):337-348. doi:10.1097/AOG.0000000000000107
Mazza GR, Youssefzadeh AC, Klar M, et al. Association of Pregnancy Characteristics and Maternal Mortality With Amniotic Fluid Embolism. JAMA Netw Open. 2022;5(11):e2242842. Published 2022 Nov 1. doi:10.1001/jamanetworkopen.2022.42842

Breaking Barriers with Amniotic Fluid Embolism Management with Interventional Radiology

Abstract Title: Unique Presentation of Diabetic Striatopathy in an Elderly Female
Background:
Diabetic striatopathy (DS), also known as hyperglycemic chorea-ballism or nonketotic hyperglycemic hemichorea-hemiballism, is a rare neurological complication predominantly seen in individuals with type 2 diabetes experiencing poorly controlled hyperglycemia. Radiologically, DS is identified by specific hyperintensities in the basal ganglia on T1-weighted MRI, and striatal hyperdensity on CT scan; a hallmark of the disease's impact on central nervous system function. Management of DS primarily focuses on rapid correction of hyperglycemia and symptomatic control of chorea. Despite strict glucose control, recurrence rates have shown to be 18.2%, making the timely diagnosis of DS a crucial component in patient management.
Case Presentation:
This report is based upon the clinical case of a 79-year-old female with a medical history of type 2 diabetes mellitus, hypertension, hyperlipidemia and dementia who presented to the emergency department (ED) with altered mental status displaying confusion, but no evidence of chorea. Preliminary workup was performed in the ED and was significant for mild tachycardia, tachypnea, and elevated blood pressure. Pertinent laboratory findings revealed hyponatremia of 130 mEq/L, hyperkalemia of 6.5mEq/L, hypocarbia with CO2 of 8mEq/L, increased anion gap of 29, elevated glucose of 796 mg/dL, increased blood urea nitrogen and creatinine of 67 mg/dL and 2.7 mg/dL. Furthermore, brain CT was performed and revealed an acute left basal ganglia hemorrhage. The patient was first admitted to the intensive care unit and then stabilized and moved to the neurological step-down unit. Repeat brain CT confirmed the diagnosis of diabetic striatopathy. Lantus was initially administered aggressively to achieve rapid glucose control and was titrated down by 5 units nightly until appropriate serum glucose was achieved. In addition, the patient was started on Glipizide, Metformin, and 10 units of Humalog sliding scale as needed. The patient's blood glucose levels showed impressive improvement during the course of her hospitalization. She was hospitalized for 10 days and her condition gradually improved. 
Discussion:
This case is a unique presentation of DS in an elderly patient with uncontrolled type 2 diabetes mellitus. Awareness of the risk factors and clinical manifestations of DS is vital for accurate assessment and effective management. Furthermore, given the likelihood of recurrence, documenting a prior diagnosis of DS is imperative for optimizing future care in patients with a history of DS.

Diabetic Striatopathy in an Elderly Female

Background: The anterior cruciate ligament (ACL) is the most commonly injured ligament of the human body. Most ACL tears occur secondary to pivot shift injuries on the planted foot. A sudden change in direction on the flexed knee with valgus stress may tear the ligament and precipitate a cascade of osseous collisions. Bone contusions on both surfaces of the knee joint, termed kissing contusions, may result. Kissing contusions are most common in the lateral compartment of the knee following an ACL tear. As energy increases, additional contusions may occur in the medial compartment. Patellotibial contusions represent a third, less common contusion pattern. This clinical vignette aims to introduce the clinical presentation and implications of patellotibial contusions. 

Case Presentation: A 22-year-old male presents to the emergency department following a fall. Upon physical examination, a positive anterior drawer sign was noted. MR imaging confirmed the diagnosis of an acute ACL tear. Three kissing contusions were of note within the knee joint, consistent with patellotibial contusion. 

Discussion: Patellotibial contusions are a less common ACL tear contusion pattern at MR imaging that usually results from an axial load such as a jump or fall. In addition to lateral and medial sided contusions, impaction of the inferior patella with the anteromedial tibial rim occurs. Not all patellotibial contusions result in the classic three pairs of kissing contusions. In fact, most patients present with five total contusions: kissing contusions laterally, kissing contusions medially, and an unpaired contusion of the anteromedial tibial plateau. One of the reasons proposed for lack of edema in the patella is that it is a sesamoid bone with a thick peripheral cortex, which may make the patella more resistant to bone marrow edema. Furthermore, patellotibial contusions are often associated with other knee injuries. While tears of the lateral meniscus are less common than those of the medial meniscus in the setting of most ACL tears, patellotibial contusions have an increased risk of tears of the posterior horn lateral meniscus. In addition, posterolateral corner (PLC) injuries are strongly associated with patellotibial contusions. As the anteromedial aspect of the knee narrows during patellotibial impaction, the posterolateral aspect of the knee joint must widen. If undetected on imaging, PLC injuries pose a greater risk of ACL reconstructing failure. Recognizing a patellotibial contusion on MR imaging will direct the physician to search for these associated injuries.


Patellotibial Contusion in an Acute Anterior Cruciate Ligament Tear

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.


A Rapidly Progressing Case in an 81-year old Female

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.
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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.

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