United States

Objectives:
1. Outline the clinical background and pathophysiological mechanisms of spur cell hemolytic anemia
2. Discuss and highlight the presentation and interventions for a case of severe cirrhosis with associated end-stage hemolytic anemia
3. Evaluate and explore factors contributing to the unexpected clinical improvement of hemolytic anemia without liver transplantation

Background:
- Spur cell hemolytic anemia was first discussed in 1964 when a peripheral blood smear from a cirrhotic patient revealed erythrocytes with irregularly shaped projections
- Commonly associated with end-stage liver disease, this condition arises from disruptions in membrane lipid metabolism which leads to formation of acanthocytes— derived from the Greek word “acantho” meaning thorn
- Spur cell hemolytic anemia is a poor prognostic indicator, often requiring a liver transplant for clinical amelioration

Case Presentation: 
A 41-year-old female patient with decompensated cirrhosis secondary to chronic alcohol use (MELD 38) presented with jaundice and laboratory evidence of new onset acute anemia

Discussion:
- Cirrhosis disrupts lipid and cholesterol metabolism in the liver, leading to the accumulation of abnormal proteins, which alters the erythrocyte membrane and increases susceptibility to hemolysis
- The exact pathophysiology remains unclear, but it is believed that these membrane alterations result in the formation of abnormal red blood cells, such as target cells and acanthocytes, which are prone to hemolysis
- Liver transplantation is the only definitive treatment for spur cell hemolytic anemia, as it directly addresses the underlying cause of the condition
- The median survival for patients diagnosed with spur cell hemolytic anemia is approximately 1.9 years, with significantly higher mortality rates in those who are unable to undergo a liver transplant
- For patients ineligible for transplant, supportive care is the mainstay of management, aimed at minimizing symptoms and improving quality of life

Conclusion: 
- Spur cell hemolytic anemia, though associated with poor prognosis, can be managed with supportive care, including blood transfusions, liver disease management (albumin, diuretics), alcohol abstinence, and nutritional support
- The effectiveness of these treatments depends on the patient’s clinical status, MELD score, and the presence of comorbidities

AMA Research Challenge 2024 

Supportive Treatment of Spur Cell Hemolytic Anemia in the Setting of Decompensated Liver Failure

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While Gastric cancer is the second leading cause of cancer-related mortality worldwide, its metastatic spread to the breast is extremely rare. This article illustrates a patient with primary diffuse-type gastric cancer which metastasized to the breast. 


A 52-year-old post-menopausal woman presented to the hospital with severe abdominal pain and constipation. Imaging showed lymphadenopathy gastric wall thickening, and a pulmonary nodule. Biopsy revealed diffuse type, poorly differentiated adenocarcinoma. A CT-PET showed hypermetabolic nodularity suspicious for primary breast malignancy versus metastatic disease. This was subsequently proven to be metastatic via immunochemistry staining. This case represents one of very few documented cases of gastric adenocarcinoma metastasizing to the breast and emphasizes the key role of immunohistochemistry in the diagnostic process.

Uncommon Pathways: A Rare Case of Breast Metastasis from Diffuse Gastric Cancer with Sporadic CDH1 Mutation


Abstract Title
A Cavernous Sinus Mass Resection Develops Into An Eye-Opening Complication

Background
Brain tumors are a rare disease in the general population, with a reported incidence of 5 – 13 out of 100,000. Cavernous sinus tumors are a subset of brain tumors and are also rare. Cavernous sinus tumors may impact internal carotid artery, cranial nerves 3 and 4, and the ophthalmic or maxillary branches of the trigeminal nerve. When tumors are located within this structure, ophthalmoplegia, proptosis, and paresthesias may develop. Management is affected by their involvement of surrounding structures and their mass effect which may cause significant pain.

Case Presentation
This is a case of a 35-year-old male with no past medical history but three months of constant headaches associated with left facial numbness and progressive diplopia. His diplopia progressed to persistent binocular diplopia in upgaze, right gaze, and down gaze. Physical exam was remarkable for left cranial nerve 3 palsy and decreased sensation to the left face. Labs were only remarkable for a cortisol of 0.5. During workup, magnetic resonance imaging of the brain demonstrated an extra-axial mass in the left Meckel’s cave, left cavernous sinus, and left middle cranial fossa. The differential diagnosis at that time included schwannoma, spindle cell tumor, lymphoma, meningioma, pituitary adenoma, and metastases. His biopsy was inconclusive. For his palsy, the patient was given a pirate patch. 
Upon progression of symptoms, he presented to the emergency department. He underwent transsphenoidal biopsy that demonstrated a spindle cell tumor. He was then discharged, and later saw neurosurgery in clinic, who scheduled him for an elective left craniotomy with mass resection. He underwent this procedure with a postoperative course complicated by a pseudomeningocele, complete ophthalmoplegia along with proptosis, inferior displacement and lagophthalmos, with severe exposure keratopathy. Ophthalmology recommended lubricant, steroid eye drops, moxifloxacin eye drops and completed a tarsorrhaphy. The patient lost vision in his left eye due to the significant exposure keratitis due to postoperative swelling of the cavernous sinus.

Discussion
Traditionally, management of ophthalmoplegia is achieved through use of lubricant eye drops and ointments. Surgical intervention can be considered depending on the severity and duration of lagophthalmos, in which a temporary vs permeant tarsorrhaphy is considered. Mild exposure keratopathy has a very good prognosis, while more severe disease can lead to permeant corneal scarring, perforation, and vision loss. Timely consultation and treatment for cavernous sinus masses are needed to prevent progression of these symptoms.


A Cavernous Sinus Mass Resection Develops Into An Eye-Opening Complication

Acquired methemoglobinemia is rare, however it could be fatal. Symptoms such as headache, fatigue, dyspnea, and syncope occur as methemoglobin levels rise, while levels above 50% can precipitate seizures, arrhythmias, coma, and even death. We are presenting a case where a middle-aged male ingested an energy drink named as “PWED super rush "for recreational purposes. Upon checking, it was noted that this drink contains isobutyl nitrite as the main ingredient that enhances sexual experience due to its vasodilatory properties.  
A 52-year-old male with past medical history of hypertension, hyperlipidemia, asthma, and alcohol use disorder presented via EMS with acute onset of lightheadedness, a near-syncopal episode shortly after drinking an energy drink followed by nausea and vomiting.  Vital signs in the ER showed significant hypoxia and otherwise unremarkable. CXR showed no findings concerning fluid overload or community acquired pneumonia. He tested negative for covid. The patient was started on nonrebreather, however, SpO2 remained in 84-88% with supplemental oxygen. To find out the cause of unexplained refractory hypoxia, blood gas analysis was obtained that showed pH of 7.357, pCO2 44, pO2 64, with methemoglobin 25.2%. Based on ABGs results and the initiation of sequence of events following ingestion of an energy drink, toxicology was consulted, and patient was started on methylene blue. He was admitted to ICU for close monitoring and after two doses of methylene blue 1mg/kg, his clinical symptoms significantly improved, successfully transitioned to nasal cannula. Repeat ABGs afterwards showed improvement in methemoglobin levels to 1.0%. His hospital course was uncomplicated, and he was discharged home in stable condition after counseling on substance use.  
Methemoglobinemia is treated with hyperbaric oxygen, methylene blue, ascorbic acid, riboflavin in high doses and exchange transfusion in refractory cases. The first line treatment-Methylene blue acts via the NADPH reductase pathway to regenerate ferrous heme and reduce further oxidation. This case emphasizes the need for a high index of suspicion for methemoglobinemia in cases of refractory hypoxia, especially in the setting of possible toxic ingestion. Point-of-care co-oximetry can quickly diagnose elevated methemoglobin levels when unavailable on ABG analysis.  Additionally, enhanced public awareness and restricting access to agents like isobutyl nitrite are needed to prevent further recreational cases that can overburden emergency departments, intensive care units, and hospitals.  


A Dangerous Rush: When Recreational Use Turns Life-Threatening​

Background: Amiodarone is a class III antiarrhythmic agent used for the management of certain cardiac arrhythmias. Although effective and relatively safe, amiodarone is associated with a spectrum of adverse effects including neurotoxicity. There have been sparse reports of drug-induced parkinsonism (DIP) as a consequence of amiodarone use; however, the mechanism and clinical implications remain an area of limited understanding and documentation. Parksinon’s disease (PD) is associated with degeneration of dopaminergic pathways in the basal ganglia, manifesting in the classic constellation of symptoms, including “pill-rolling” tremors. These dopaminergic pathways can also be disrupted by certain medications, notably antipsychotics, antiemetics, and antiepileptics, which may mimic the symptoms of PD.

Case Presentation: 92 year-old female with a past medical history of atrial fibrillation, hypothyroidism, heart failure, COPD, and dementia presented with resting tremors of the bilateral hands for 2 months. Pt endorsed no new bradykinesia, loss of balance, or muscle stiffness. Denied any fever, chills, myalgia, cough, dysuria, nausea, vomiting, diarrhea, or weight gain or loss. Physical exam demonstrated isolated bilateral resting tremors, with an otherwise unremarkable neurologic exam. There was no muscle rigidity, cerebellar dysfunction, motor/sensory deficits, or bradykinesia compared to baseline. Vital signs were normal and labwork including TSH, RPR, and vitamins B12 and B9 were unremarkable. Following review of the patient’s medication regimen, we reduced amiodarone dosing from 200mg to 100mg due to concern for neurotoxicity rather than late-onset PD. On follow up at 2 weeks and 1 month, resting tremors resolved completely and the patient had no further concerns.

Discussion: Despite the established neurotoxicity of amiodarone, reports of amiodarone-induced Parkinsonism remain sparse. Here, we present a case of Parkinsonism occurring in a patient receiving long-term amiodarone therapy. Through this case report, we aim to broaden understanding of potential neurotoxic effects of amiodarone and emphasize the importance of recognizing drug-induced parkinsonism (DIP). DIP is a reversible form of Parkinsonism caused by the blockade of dopamine receptors or interference with dopamine transmission in the basal ganglia, mimicking the dopaminergic deficiency seen in PD. Misdiagnosing DIP as Parkinson's disease could lead to inappropriate treatment, potentially causing harm. Hence, clinical vigilance is warranted in patients receiving long-term therapy with amiodarone. 


Amiodarone-Induced Parkinsonism: A Case Report Highlighting Neurotoxicity and Clinical Vigilance in Long-Term Therapy

Abstract Title
Atrial Fibrillation Presenting as Renal Infarction
Background
Renal infarction is caused by occlusion of renal artery or its branches, due to emboli from cardiac sources or in situ thrombosis. It presents as sudden flank or abdominal pain, with labs showing elevated LDH, hematuria, proteinuria. Prompt diagnosis is crucial to initiate timely revascularization therapies.

Case Presentation
A 78-year-old male with past medical history of hypertension, diabetes mellitus, hyperlipidemia, COPD, meningioma presented to the emergency department with complaints of pain in the left flank and umbilical region which started gradually over the past few days and was described as dull and non-radiating. Physical evaluation revealed stable vital signs with hypertension, chronic normocytic anemia, irregularly irregular pulse, Left costovertebral angle (CVA) tenderness, bilateral chronic hand tremors. No focal neurological deficits. Denies fever, nausea, vomiting, diarrhea, constipation, anorexia, alcohol use, changes with meal, similar pain, incontinence, dysuria, hematuria, changes with urine output, urinary frequency, chest pains, palpitations, syncope, orthopnea, PND, edema. Initial Diagnostic workup included negative troponin, COVID-19/flu/RSV tests, and a chest X-ray showing mediastinal widening. Further investigations with a CT angiography chest ruled out aortic aneurysm or dissection but noted a region of low density in the left kidney parenchyma suggestive of multiple cortical infarcts. Urinalysis was positive for microscopic blood and 1 RBC. An EKG demonstrated new-onset atrial fibrillation (Afib). Cardiology consulted recommended to start anticoagulation therapy for atrial fibrillation and electrophysiology with no immediate plan for cardioversion. Later, CTAP with and without contrast show findings consistent with multiple acute renal infarcts (ARI) in the left kidney. Urology was consulted and determined no surgical intervention was indicated, recommending anticoagulation to prevent further complications from kidney infarcts. The patient was educated about his condition and discharged with appropriate outpatient follow-up.

Discussion
ARI is potentially serious medical condition but its clinical presentation of is often
nonspecific, leading to delays in diagnosis and treatment. Autopsy studies suggest the incidence of renal infarction is 14 per 1000, or 1.4%. Other retrospective studies of ER admissions found an incidence of 0.004% to 0.007%. In our case, the patient's abdominal pain with no previous history of arrhythmias prompted further evaluation, revealing new-onset AFib on EKG and subsequent diagnosis of renal infarction on imaging. Afib can cause thrombotic complication to any body part, most commonly being ischemic stroke. Clinicians should suspect other systemic embolization phenomena in patients with Afib.


Atrial Fibrillation Presenting as Renal Infarction: A Case Report

Introduction. Variegate Porphyria (VP) is a rare autosomal dominant disorder characterized by mutations in the protoporphyrinogen oxidase (PPOX) gene, leading to diminished enzymatic activity, critical for heme biosynthesis. The clinical manifestations of VP are highly variable, with some patients exhibiting significant neurovisceral symptoms and others presenting with cutaneous photosensitivity, many with a combination of both. This variability reflects the disease's ability to often go undiagnosed, especially in pediatric populations, where the disease is not frequently seen in clinical practice.

Methods. This case study focuses on a 12-year-old male who presented with persistent photosensitivity, painful blistering, severe abdominal pain, and dark-colored urine. Despite normal renal and liver serology testing, elevated porphyrin levels in both urine and stool samples supported the presumptive diagnosis of VP, though the diagnosis was significantly delayed resulting in multiple unnecessary hospitalizations secondary to recurrent acute attacks.

Discussion. The complexity of VP, particularly in pediatric patients, often results in diagnostic delays due to the vague symptoms that oftentimes represent other medical conditions. Our case emphasizes the importance of a thorough clinical evaluation, family history, and awareness of this rare disorder. Management strategies, including sun protection and beta-carotene supplementation, were implemented to mitigate the risk of acute attacks.

Conclusion. This study emphasizes the need for increased awareness and understanding of VP, aiming to promote earlier diagnoses and improve patient outcomes through timely and appropriate interventions.

Case Study: Navigating the Complexities of Variegate Porphyria

Anti-TNF therapy has revolutionized the treatment of various autoimmune conditions. However, it has also been associated with an increased risk of bacterial infections. To our knowledge, no case of Acinetobacter baumannii bacteremia has been reported with adalimumab treatment.

A 63-year-old female with generalized pustular psoriasis (GPP) was referred to the ED by her dermatologist for worsening rash, severe weakness, and fatigue. Symptoms began a week prior and acutely worsened after adalimumab infusion a day before hospital presentation. This was her second infusion, the first had been two weeks earlier. In the ED, her vital signs and labs were unremarkable. However, she had chills, prompting the collection of two blood cultures at different sites, which were both positive for A. baumannii. She had no clear infection source. Infectious disease (ID) was consulted, and she was started on ampicillin-sulbactam. Her weakness and fatigue improved shortly after starting antibiotics. A transthoracic echocardiogram showed no vegetations, and repeat blood cultures were negative. Dermatology attributed her worsening rash to a GPP flare, and spesolimab was recommended. Her medical records showed a positive Quantiferon-TB Gold, with a low mitogen-nil value and a chest X-ray showing no evidence of past or current tuberculosis infection. ID suggested the result was a false positive, but a subsequent positive T-spot confirmed latent tuberculosis. Biologic treatment was suspended until completing one month of latent tuberculosis treatment. Outpatient ID planned to initiate once-weekly isoniazid plus rifapentine. Meanwhile, acitretin was started, with dermatology follow-up scheduled for one month later to begin spesolimab after latent tuberculosis treatment. She was discharged on ciprofloxacin to complete her antibiotic course.

Acinetobacter baumannii, part of the ESKAPE organisms, is predominantly associated with healthcare-related infections and known for developing antibiotic-resistant strains. In the US, community-acquired A. baumannii cases are rare, with most being hospital-acquired infections. Moreover, shaking chills is an independent predictor of bloodstream infection. Given that blood is generally sterile, there is a low likelihood it was a contaminant. This case highlights the importance of clinical exam findings to maintain a high index of suspicion for severe infections such as bacteremia in immunocompromised individuals, and the necessity for physicians to broaden differentials to include rare organisms even in the absence of recent clinical exposure. Fortunately, the isolate was pan-sensitive, as multidrug-resistant A. baumannii infections can be life-threatening. Additionally, we are not suggesting that the patient's symptoms were solely due to bacteremia; reactivation of latent tuberculosis may have contributed.



Community Acquired Acinetobacter baumannii Bacteremia After Receiving an Infusion of Adalimumab

Title: Delayed Diagnosis of Disseminated Coccidioidomycosis Due to Misdiagnosis
of Lymphoma: A Case Report.

Background:
Coccidioidomycosis, or valley fever, is a fungal infection caused by inhaling Coccidioides imitis/posadasii. These fungi are endemic to the southwestern USA, Mexico, and Central and South America. Incidence has been rising, likely due to population growth, climatechange, and increased awareness. The infection presents diversely, with most cases being asymptomatic. About one-third develop pulmonary illness, and a small percentage experience dissemination to other body areas, commonly to skin, bone, and CNS, particularly in immunocompromised individuals and certain ethnic groups.

Case presentation:
A 39-year-old Hispanic female with a history of non-Hodgkin's lymphoma and treated tuberculosis presented with painful ulcerative skin lesions. Initial labs showed mild leukocytosis, microcytic anemia, hypokalemia, and hypoalbuminemia. Imaging revealed bilateral pulmonary infiltrates, mediastinal adenopathy, and extensive lymphadenopathy. A skin biopsy report from Mexico indicated non-Hodgkin lymphoma, but no treatment was initiated at the time of diagnosis. A neck lymph node biopsy revealed fungal spherules and hyphae, consistent with coccidioidomycosis. Serology confirmed high titer IgG for Coccidioidomycosis. Further testing ruled out other conditions, including lymphoma. The patient started high-dose fluconazole. A lumbar puncture showed positive IgG for Coccidiomycosis in CSF, indicating CNS involvement. A repeated skin biopsy was negative for lymphoma, confirming disseminated coccidioidomycosis and ruling out
lymphoma diagnosis. The patient was discharged on oral fluconazole and prophylactic TMP-SMX. Two months later, she was readmitted with worsening weakness and altered mentation. Despite adequate treatment, the imaging showed new lytic lesions in the left scapula and T2 body. Repeated lumbar puncture confirmed CNS involvement. Her condition improved with continued antifungal. Not candidate for amphotericin B.

Discussion:
This case highlights the difficulty in diagnosing coccidioidomycosis, which is often
mistaken for other conditions. The patient's initial misdiagnosis of lymphoma while
undergoing care in Mexico led to a delay in appropriate treatment. Initially, treatment focused on a superimposed skin infection, assuming the underlying cause was NHL. However, even after diagnosing pulmonary coccidioidomycosis, the misdiagnosis of NHL persisted until a repeat skin biopsy ruled it out. This case highlights the challenge of differentiating reactive inflammatory changes from malignancy, which can delay a definitive diagnosis. The diagnosis was eventually confirmed through lymph node biopsy and subsequent serology testing, the most appropriate diagnostic methods. The initial treatment with fluconazole was appropriate as azoles remain the gold standard for therapy. The case underscores the need for timely and accurate diagnosis to initiate appropriate antifungal therapy and prevent complications.

Delayed Diagnosis of Disseminated Coccidioidomycosis Due to Misdiagnosis of Lymphoma: A Case Report

Background: 
Infectious myositis, a rare but significant inflammatory muscle disease, involves infection of skeletal muscle tissue, typically caused by bacteria, viruses, fungi, and parasites. Staphylococcus aureus is the most common bacterial pathogen. Symptoms range from localized pain and swelling to systemic manifestations like fever and malaise. Early recognition and management are crucial to prevent complications like abscess formation, sepsis, and chronic disability 1. Here, we detail the case of a 21-year-old female with right lower extremity (RLE) pain and swelling who developed infectious myositis and an abscess in the anterior leg compartment despite having no skin defects.

Case Presentation:
A 21-year-old female with systemic lupus erythematosus (SLE) presented to the ED after a mechanical fall with RLE pain, swelling, and fevers for two days. Physical examination revealed RLE swelling and tenderness without overlying skin findings. Lab results showed creatine phosphokinase (CPK) 125, C-reactive protein (CRP) 12.3, erythrocyte sedimentation rate (ESR) 72, and D-dimer 4.19 FEU. Initial CT of the RLE indicated subcutaneous edema, muscle atrophy, and fascial plane blurring, suggesting myositis. A RLE Doppler ultrasound was negative for thromboembolic processes. Given symptom worsening despite a negative workup, repeat CT of the RLE with contrast was obtained five days later which revealed a complex, multiloculated fluid collection indicative of an abscess within the anterior compartment. She was treated with IV vancomycin and underwent incision and drainage of the RLE. Intraoperative cultures grew methicillin-resistant S. aureus. Despite persistent fevers, extensive workup was unrevealing, and blood cultures were negative. At discharge, she was afebrile with improved RLE symptoms and uneventful wound healing.

Discussion:
This case highlights an atypical presentation of infectious myositis in an immunosuppressed patient following a mechanical fall without any obvious historical or exam findings of skin lacerations. Differential diagnosis included myositis, pyomyositis, deep vein thrombosis, hematoma, and compartment syndrome. Despite high suspicion for myositis, normal CPK levels, lack of leukocytosis, and unrevealing CT imaging complicated diagnosis and management. Elevated ESR and CRP levels were ambiguous due to her SLE history. CT imaging, although useful, has limitations in detecting early or subtle inflammatory changes in soft tissue and persistence of symptoms necessitated further investigation. This case underscores the importance of maintaining a high index of clinical suspicion for infectious myositis and the need for repeat imaging or alternative diagnostic modalities 2 when initial tests are inconclusive, given the risk of serious complications like sepsis, osteomyelitis, and necrotizing fasciitis with delayed treatment.

Diagnostic Dilemma: Atypical Presentation of Infectious Myositis in a Young Immunocompromised Female Following a Mechanical Fall

Abstract Title
Drug Induced ANCA Vasculitis with Long-standing Tolerance to Hydralazine.
Background
Anti-neutrophil cytoplasmic antibodies (ANCA) associated vasculitis has a varied presentation and positive ANCA antibodies are commonly found in granulomatosis with polyangiitis, microscopic polyangiitis (MPA), renal-limited vasculitis, and drug-induced vasculitis. Specific ANCA antibodies, anti-protease 3 (PR3) and myeloperoxidase (MPO), can help differentiate between the various vasculitides with MPO-ANCA most closely associated with MPA, renal limited, and drug induced. Kidney biopsies most frequently demonstrate necrotizing crescentic glomerulonephritis. Symptoms vary depending on the organ system involved but consistently involve signs of systemic inflammation and end organ damage.
Case Presentation
This case presents a 67-year-old male with history of chronic obstructive pulmonary disease (COPD), chronic kidney disease stage IV (CKD IV), and hypertension presenting with newfound hematuria, acute respiratory failure, and altered mentation. He was found to be hypertensive and ultimately required intubation for airway protection. On review of home medications, antihypertensive regimen includes hydralazine, carvedilol, and nifedipine.
Urinalysis was significant for significant hematuria and proteinuria. Lab work was significant for elevated creatinine, blood urea nitrogen, and respiratory acidosis. Rheumatologic work up revealed positive titers for antinuclear antibodies 1:640, ANCA, myeloperoxidase antibodies at 35 U/mL, and weakly positive anti-histone antibodies at 1.3 units. Work up negative for C3 abnormalities, anti-glomerular basement membrane antibodies, and anti-PR3 titers. Renal biopsy showed pauci-immune necrotizing crescentic glomerulonephritis. 
A presumptive diagnosis of drug-induced ANCA-associated MPA was made and methylprednisolone was started for 3 days. The patient was transitioned to high dose prednisone with appropriate taper until outpatient follow up for reevaluation. The patient also started on a two part rituximab infusion occurring 14 days apart. The patient’s renal and pulmonary function responded well to the steroid and rituximab treatment. The patient was successfully stabilized and discharged with appropriate prophylaxis and scheduled appointments for the outpatient Rituximab infusion and nephrology follow up.
Discussion
ANCA-associated vasculitis has a wide spectrum of symptom presentations. Thorough medication reconciliation and work up for any new acute kidney injury should include investigation into autoimmune pathologies when occurring with widespread organ involvement. Drug -induced ANCA vasculitis can present in a patient even after years of taking high risk medications, and as such previous tolerance should not exclude a new drug reaction. Furthermore, the proper coordination of inpatient services with outpatient therapy is required to promote optimum patient outcomes.


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