Abstracts from the First Annual Scholarly Day
The Department of Medical Education at Baylor University Medical Center at Dallas hosted its first annual Scholarly Day to showcase the research efforts of medical students, residents, and fellows. Hania Wehbe-Janek, PhD, vice president for academic research integration for Baylor Scott and White Health Central Texas, shared the plenary session, “Building a Culture of Research at Academic Medical Centers: Impact on Medical Education and Recommended Practices.” Stuart Black, MD, John Fordtran, MD, Ronald C. Jones, MD, Michael A. Ramsay, MD, William C. Roberts, MD, and Hania Wehbe-Janek, PhD, served as judges of the posters and the oral presentations. Winners in each category were as follows: best fellow poster, “Diet pattern and cardiovascular disease among women with type 2 diabetes mellitus,” by Hyun Joon Shin, MD; best resident poster, “Oral squamous cell carcinoma: current concepts in imaging, staging, and fibular osteocutaneous free-flap reconstruction,” by R. Evans Heithaus, MD; best medical student poster, “Neuromyelitis optica,” by Elizabeth Coffee; and best oral presentation, “Transarterial chemoembolization with smaller beads: midterm clinical outcomes,” by R. Evans Heithaus, MD. Overall, participants in the inaugural event presented more than 40 posters and six oral presentations. This article reprints a selection of the abstracts.
Cutaneous metastasis from hepatocellular carcinoma to the abdomen Jamil Alsahhar, MD (e-mail: [email protected])
Hepatocellular carcinoma (HCC) is the sixth most prevalent cancer and the third leading cause of cancer mortality worldwide. Patients with cirrhosis are at highest risk of developing this malignant disease. Metastases are most commonly seen in the lung, abdominal lymph nodes, and bones. Cutaneous metastases of HCC are uncommon. We report a case of a 54-year-old Caucasian man with a history of HCC treated with six rounds of transarterial chemoembolization followed by CyberKnife therapy, as well as a history of hepatitis C cirrhosis and diabetes mellitus, who presented with complaints of abdominal rash and pain. Examination revealed multiple erythematous, firm, well-circumscribed, dry, subcutaneous nodules surrounding the umbilicus and left abdomen. The nodules were tender to palpation. The patient was initially started on antibiotics, as the rash was concerning for cellulitis. A histopathological analysis of the nodules was consistent with high-grade adenocarcinoma of hepatobiliary origin. Electron beam therapy was utilized for palliation. HCC Proc (Bayl Univ Med Cent) 2015;28(3):321–324
often metastasizes to the lungs, abdominal lymph nodes, and bones. A literature review showed that cutaneous metastasis of HCC to the abdomen is rare, with most cases occurring at sites of previous biliary drains or biopsies. Cutaneous nodules presenting in patients with HCC or hepatobiliary malignancies should be biopsied to assess for possible metastases. Formalized resident training in code blue execution in a simulation lab improves immediate post-code survival Adan Mora Jr., MD, Benjamin Bijas, MD, Britton Smialek, MD, Bradley Christensen,* Jennifer Duewall, MD, and Cristie Columbus, MD (*e-mail: [email protected])
Although residents complete advance cardiac life support (ACLS) training, they rarely receive formal instruction in the practical elements of leading and executing a code blue in a hospital setting. This study tested whether code simulation could facilitate residents’ formal training and improve code blue execution and postcode outcomes. Internal medicine residents at Baylor University Medical Center were trained for a 10-month period in a simulation lab on a 3G version SimMan. Residents were exposed to progressively more challenging arrhythmias and code scenarios in which ACLS was implemented and were observed by internal medicine and critical care faculty. The hospital’s code blue data for a 12-month historical period were then compared with the 10-month intervention period during which simulation training was implemented. There were 107 codes (an average of 8.9 per month) during the historical control period, with immediate postcode survival noted in 72 codes (67.3%), and 180 codes (an average of 16.4 per month) during the intervention period, with immediate postcode survival noted in 128 codes (71.1%). The hospital census during the 22-month study period was stable. Twenty-five patients (23.4%) survived to discharge in the control period, compared with 40 patients (22.2%) in the intervention period (P = .82; NS). While formal resident training in code execution in a simulation lab may improve immediate postcode survival in hospitalized patients, the trend seen in immediate postcode survival did not translate to discharge. This may be a result of improved ACLS execution of patients in whom successful resuscitation would not have previously been achieved. Further study is needed to determine whether or not the observed trend in immediate postcode survival is significant. 321
Neuromyelitis optica heralded by intractable hiccups, nausea, and vomiting Elizabeth Coffee* and Chaouki Khoury, MD (*e-mail: coffee@medicine. tamhsc.edu)
A 19-year-old woman presented to the emergency department with vertigo, dysphonia, dysphagia, right tongue deviation, left hemiataxia, left hemiparesis, and vertical oscillopsia. Upon further questioning, it was revealed that the patient had experienced intractable hiccups, nausea, and vomiting (IHN) 3 weeks earlier that was treated as an acute gastroenteritis. She then developed wheezing, which was treated as reactive airways disease, and progressed to the previously listed symptoms. On admission she was found to have a single right dorsal medullary lesion on magnetic resonance imaging (MRI) and was later determined to be seropositive for aquaporin-4 antibody. Thus, a diagnosis of neuromyelitis optica (NMO) was made. NMO is a rare inflammatory demyelinating disease of the central nervous system. Current diagnostic criteria for NMO require both optic neuritis and acute myelitis and at least two of the following: contiguous spinal cord lesion, MRI that does not meet criteria for multiple sclerosis, and seropositive NMO IgG. However, IHN is increasingly recognized as an early presentation of NMO that distinguishes it from multiple sclerosis. This distinction is crucial in order to avoid potentially harmful treatments for patients with NMO mistaken for multiple sclerosis. This report suggests that an indepth assessment of IHN, including a brain MRI, is needed for a prompt and accurate diagnosis of NMO. Continuous renal replacement therapy to treat severe metformin-induced lactic acidosis Jeffrey Klein, MD,* and Harold Szerlip, MD (*e-mail: Jeffrey.Klein@ baylorhealth.edu)
Chronic metformin ingestion rarely causes lactic acidosis, but the risk is increased in chronic kidney disease. We present a case of severe lactic acidosis in a patient with diabetes treated with metformin, complicated by acute on chronic renal failure. A 54-year-old African American man with hypertension, insulindependent diabetes mellitus, chronic kidney disease (serum creatinine 1.5 mg/dL), and bilateral above-the-knee amputations presented to the emergency department with a 3-day history of lethargy and pruritus. The patient was taking insulin, simvastatin, and metformin (500 mg twice a day). Initial laboratory evaluation revealed leukocytosis (11,000/mm3) and severe renal failure (blood urea nitrogen 89 mg/dL; creatinine, 10.2 mg/dL), as well as a potassium level of 6.2 mmol/L, anion gap of 24, and glucose of 198 mg/dL. His lactate was 11 mmol/L with an arterial blood gas of pH 6.5 and CO2 0.05) and the angle between FNV and LTV (40.2 ± 9.7 vs 38.3 ± 9.6, P > 0.05) were not significantly different between groups. In conclusion, the lesser trochanteric retroversion is significantly increased in patients with psoas impingement compared with patients with asymptomatic hips. July 2015
Beyond Typhoid Mary: the sophistication of Salmonella typhi Charis Santini,* Curtis R, Mirkes, DO, and Cedric Spak, MD (*e-mail: santini@ medicine.tamhsc.edu)
As resistant strains of Salmonella typhi are increasing worldwide, a clinician in the United States may find it more difficult to treat typhoid fever in returning travelers. This case demonstrates the variability within which typhoid fever can present. A 20-yearold formerly healthy man presented with an 18-day history of intermittent fever and myalgia. The fever spiked to approximately 104°F in the late afternoons. Associated symptoms included chills, headache, weakness, nausea/vomiting, and occasional loose stools. The patient returned from Bangladesh 5 days prior to symptom onset. He denied contact with contaminated food, sick contacts, bloody diarrhea, constipation, or rashes. His laboratory results were significant for a white blood cell count of 4.7 × 109/L with 37% bands, thrombocytopenia (109 × 109/L), hyponatremia (129 mmol/L), and elevated transaminases (aspartate transaminase [AST] and alanine transaminase [ALT], 157 and 138 U/L). Cultures were obtained and the patient was placed empirically on vancomycin and piperacillin/tazobactam. Serum cultures were positive after 48 h for gram-negative rods. After 3 days of treatment, the patient’s symptoms did not improve. His liver transaminases continued to increase to a maximum AST/ALT of 301/245 U/L. Also, his hemoglobin and hematocrit levels dropped, with laboratory values consistent with intravascular hemolysis. The patient improved on ceftriaxone. Culture growth further revealed S. typhi group D with intermediate sensitivity to ciprofloxacin. He was discharged on oral antibiotics. This case of typhoid fever was not the straightforward picture of enteritis, bloody diarrhea/constipation, and salmon-colored macules. Once typhoid fever is diagnosed, a new difficulty arises with the changing resistance patterns; selecting appropriate antibiotic therapy is of the utmost importance. Fluoroquinolones for 7 to 10 days is the recommended treatment. However, in certain regions, including South Asia, fluoroquinolone resistance is becoming more prevalent. Therefore, in cases of known fluoroquinolone resistance, recommendations promote the use of a third-generation cephalosporin or azithromycin. Delaying appropriate therapy increases the risk of relapse and can result in a mortality rate of 10% to 20%. Acute enlargement and dysfunction of the exocrine glands of the head Avery Smith, MD,* and Alan Hise (*e-mail: [email protected])
We present a case of immunoglobulin G4-related disease (IgG4-RD), a recently recognized syndrome. Diagnosis generally requires elevated serum IgG4 levels, biopsy-proven elevation in IgG4, and a rapid response to steroid treatment. A 23-yearold Ethiopian man presented with 2 weeks of facial swelling of his upper eyelids, submandibular area, and cheeks. He endorsed visual impairment, nasal stuffiness, dry mouth/eyes, and odynophagia. Laboratory values were normal except for eosinophilia. Magnetic resonance imaging of the head demonstrated significant bilateral lacrimal gland swelling and buccal lymphadenopathy. The patient began to have a sensation that his throat was “closing up” so was given high-dose intravenous dexamethasone.
Abstracts from the First Annual Scholarly Day
323
Subsequently, he had dramatic improvement. A bilateral lacrimal gland biopsy showed many B and T lymphocytes mixed with eosinophils. The lymphocytes were prominently positive for IgG. Only 1% of the lymphocytes were positive for IgG4, but his serum IgG4 level was slightly elevated at 97 mg/dL. He was transitioned to oral prednisone. After completing the steroid taper, his facial features were normal, eosinophilia resolved, and he was asymptomatic. He has not had a relapse to date. Enlargement of the lacrimal and salivary glands, known as Mikulicz’s disease, was once considered a subtype of Sjögren syndrome. This disease has since been reclassified as falling within the IgG4-RD spectrum. This case had many features of IgG4-RD but did not meet strict diagnostic criteria. However, suspicion for IgG4-RD remains high based upon his presentation, response to treatment, and exclusion of other diagnoses. Antinuclear antibody and anti-Ro/SSA and anti-La/SSB antibodies were negative. The marked peripheral eosinophilia and prominent eosinophils in his lacrimal gland biopsy suggested an allergic etiology. However, serum IgE levels were normal. Thus, his peripheral and biopsy-proven eosinophilia was attributed to a reactive process secondary to the recruitment of T lymphocytes, a well-recognized feature of IgG4-RD. The patient’s dramatic response to dexamethasone and the eventual resolution of his dry eyes/mouth support IgG4-RD over Sjögren syndrome. Thus, it appears that intensive steroid treatment can markedly decrease peripheral and glandular IgG4 levels, and this may have accounted for his negative results. A rare case of recurrent focal myositis and an associated coagulopathy Avery Smith, MD,* and Carolyn Quan, MD (*e-mail: Avery.Smith@ baylorhealth.edu)
Focal myositis is a rare disorder with less than 50 cases reported to date. It typically presents as localized swelling, pain, and skin discoloration over a muscle group of an extremity. We present a case of recurring focal myositis complicated by an autoimmune coagulopathy. A 74-year-old African American man presented to the emergency department with a 3-day history of right calf swelling, dark discoloration, and pain, which started abruptly without inciting trauma. He denied fever, chills, or night sweats, but did note a similar presentation 2 months earlier in his left upper extremity. Based on magnetic resonance imaging, myositis was diagnosed, and the patient was treated with antibiotics despite negative cultures. He improved but then returned with the right calf pain. Laboratory results showed markedly positive antinuclear antibody, positive anti-centromere antibody, and an elevated erythrocyte sedimentation rate/C-reactive protein. Surgical muscle biopsy showed necrosis and muscle regeneration but no infection, dermatomyositis, polymyositis, or inclusion body myositis. The procedure was complicated by significant bleeding. Studies showed an isolated prolongation of the partial thromboplastin time, which led to the diagnosis of a factor VIII inhibitor.
324
Electromyography performed to evaluate for subclinical weakness had unremarkable results. However, his myositis recurred at the electromyography insertion sites. He was then treated with high-dose prednisone, rituximab, and cyclophosphamide. He improved without further bleeding or recurrence of his myositis. This case is unique because the patient experienced recurrent focal myositis as well as the development of a rare autoimmune-driven coagulopathy. Heffner’s theory regarding micro-trauma as an inciting factor was supported by this patient’s recurrent myositis following the electromyography study. Focal myositis is an atypical, rheumatologic syndrome that can mimic infections of other rheumatologic entities. It is presumably secondary to an autoimmune response against a muscular antigen, but the specific antibody remains unknown, with negative myositis panels, antistriated muscle antibody, and paraneoplastic panel. Delayed treatment for herpes zoster resulting in disabling plexopathy in the immunosuppressed patient Anwar Y. Zaman, MD,* Sridevi Mukkamala, MD, and Matt Bayazitoglu, MD (*e-mail: [email protected])
Here we report two cases of adults on chronic immunosuppressive therapy who were diagnosed with herpes zoster and treated with oral steroids alone. Both patients developed focal weakness in the affected limb. A 63-year-old man on chronic immunosuppression after liver transplantation presented to his primary care physician with a left lower extremity vesicular rash and pain. He was initially started on oral steroids and within 5 weeks developed left lower extremity weakness with foot drop. He subsequently was admitted to the hospital and started on antiviral treatment. The second patient was a 66-yearold man with rheumatoid arthritis on chronic immunosuppression who presented to his primary care physician with a right thigh vesicular rash and pain. He was started on oral steroids and 1 month later developed progressive right lower extremity weakness. Nerve conduction studies and electromyography were performed on the patients more than a month after onset of symptoms. Each patient had electrodiagnostic evidence of lumbosacral plexopathy with significant motor denervations that matched clinical findings. Lumbosacral plexopathy with motor involvement occurring in the context of herpes zoster is rare and underrecognized. The patients each developed severe weakness with minimal recovery at 2-month follow-up. The patients did not receive antiviral treatment at the time of initial rash presentation. The delay in antiviral treatment may have contributed to dissemination of the infection and extensive motor involvement. In conclusion, patients on chronic immunosuppressive therapy can be at higher risk of developing plexopathy or polyradiculopathy secondary to herpes zoster. In this patient population, early disease recognition and treatment with antiviral medication may help prevent plexopathy and subsequent disability.
Baylor University Medical Center Proceedings
Volume 28, Number 3
The Department of Medical Education at Baylor University Medical Center at Dallas hosted its first annual Scholarly Day to showcase the research efforts of medical students, residents, and fellows. Hania Wehbe-Janek, PhD, vice president for academic research integration for Baylor Scott and White Health Central Texas, shared the plenary session, “Building a Culture of Research at Academic Medical Centers: Impact on Medical Education and Recommended Practices.” Stuart Black, MD, John Fordtran, MD, Ronald C. Jones, MD, Michael A. Ramsay, MD, William C. Roberts, MD, and Hania Wehbe-Janek, PhD, served as judges of the posters and the oral presentations. Winners in each category were as follows: best fellow poster, “Diet pattern and cardiovascular disease among women with type 2 diabetes mellitus,” by Hyun Joon Shin, MD; best resident poster, “Oral squamous cell carcinoma: current concepts in imaging, staging, and fibular osteocutaneous free-flap reconstruction,” by R. Evans Heithaus, MD; best medical student poster, “Neuromyelitis optica,” by Elizabeth Coffee; and best oral presentation, “Transarterial chemoembolization with smaller beads: midterm clinical outcomes,” by R. Evans Heithaus, MD. Overall, participants in the inaugural event presented more than 40 posters and six oral presentations. This article reprints a selection of the abstracts.
Cutaneous metastasis from hepatocellular carcinoma to the abdomen Jamil Alsahhar, MD (e-mail: [email protected])
Hepatocellular carcinoma (HCC) is the sixth most prevalent cancer and the third leading cause of cancer mortality worldwide. Patients with cirrhosis are at highest risk of developing this malignant disease. Metastases are most commonly seen in the lung, abdominal lymph nodes, and bones. Cutaneous metastases of HCC are uncommon. We report a case of a 54-year-old Caucasian man with a history of HCC treated with six rounds of transarterial chemoembolization followed by CyberKnife therapy, as well as a history of hepatitis C cirrhosis and diabetes mellitus, who presented with complaints of abdominal rash and pain. Examination revealed multiple erythematous, firm, well-circumscribed, dry, subcutaneous nodules surrounding the umbilicus and left abdomen. The nodules were tender to palpation. The patient was initially started on antibiotics, as the rash was concerning for cellulitis. A histopathological analysis of the nodules was consistent with high-grade adenocarcinoma of hepatobiliary origin. Electron beam therapy was utilized for palliation. HCC Proc (Bayl Univ Med Cent) 2015;28(3):321–324
often metastasizes to the lungs, abdominal lymph nodes, and bones. A literature review showed that cutaneous metastasis of HCC to the abdomen is rare, with most cases occurring at sites of previous biliary drains or biopsies. Cutaneous nodules presenting in patients with HCC or hepatobiliary malignancies should be biopsied to assess for possible metastases. Formalized resident training in code blue execution in a simulation lab improves immediate post-code survival Adan Mora Jr., MD, Benjamin Bijas, MD, Britton Smialek, MD, Bradley Christensen,* Jennifer Duewall, MD, and Cristie Columbus, MD (*e-mail: [email protected])
Although residents complete advance cardiac life support (ACLS) training, they rarely receive formal instruction in the practical elements of leading and executing a code blue in a hospital setting. This study tested whether code simulation could facilitate residents’ formal training and improve code blue execution and postcode outcomes. Internal medicine residents at Baylor University Medical Center were trained for a 10-month period in a simulation lab on a 3G version SimMan. Residents were exposed to progressively more challenging arrhythmias and code scenarios in which ACLS was implemented and were observed by internal medicine and critical care faculty. The hospital’s code blue data for a 12-month historical period were then compared with the 10-month intervention period during which simulation training was implemented. There were 107 codes (an average of 8.9 per month) during the historical control period, with immediate postcode survival noted in 72 codes (67.3%), and 180 codes (an average of 16.4 per month) during the intervention period, with immediate postcode survival noted in 128 codes (71.1%). The hospital census during the 22-month study period was stable. Twenty-five patients (23.4%) survived to discharge in the control period, compared with 40 patients (22.2%) in the intervention period (P = .82; NS). While formal resident training in code execution in a simulation lab may improve immediate postcode survival in hospitalized patients, the trend seen in immediate postcode survival did not translate to discharge. This may be a result of improved ACLS execution of patients in whom successful resuscitation would not have previously been achieved. Further study is needed to determine whether or not the observed trend in immediate postcode survival is significant. 321
Neuromyelitis optica heralded by intractable hiccups, nausea, and vomiting Elizabeth Coffee* and Chaouki Khoury, MD (*e-mail: coffee@medicine. tamhsc.edu)
A 19-year-old woman presented to the emergency department with vertigo, dysphonia, dysphagia, right tongue deviation, left hemiataxia, left hemiparesis, and vertical oscillopsia. Upon further questioning, it was revealed that the patient had experienced intractable hiccups, nausea, and vomiting (IHN) 3 weeks earlier that was treated as an acute gastroenteritis. She then developed wheezing, which was treated as reactive airways disease, and progressed to the previously listed symptoms. On admission she was found to have a single right dorsal medullary lesion on magnetic resonance imaging (MRI) and was later determined to be seropositive for aquaporin-4 antibody. Thus, a diagnosis of neuromyelitis optica (NMO) was made. NMO is a rare inflammatory demyelinating disease of the central nervous system. Current diagnostic criteria for NMO require both optic neuritis and acute myelitis and at least two of the following: contiguous spinal cord lesion, MRI that does not meet criteria for multiple sclerosis, and seropositive NMO IgG. However, IHN is increasingly recognized as an early presentation of NMO that distinguishes it from multiple sclerosis. This distinction is crucial in order to avoid potentially harmful treatments for patients with NMO mistaken for multiple sclerosis. This report suggests that an indepth assessment of IHN, including a brain MRI, is needed for a prompt and accurate diagnosis of NMO. Continuous renal replacement therapy to treat severe metformin-induced lactic acidosis Jeffrey Klein, MD,* and Harold Szerlip, MD (*e-mail: Jeffrey.Klein@ baylorhealth.edu)
Chronic metformin ingestion rarely causes lactic acidosis, but the risk is increased in chronic kidney disease. We present a case of severe lactic acidosis in a patient with diabetes treated with metformin, complicated by acute on chronic renal failure. A 54-year-old African American man with hypertension, insulindependent diabetes mellitus, chronic kidney disease (serum creatinine 1.5 mg/dL), and bilateral above-the-knee amputations presented to the emergency department with a 3-day history of lethargy and pruritus. The patient was taking insulin, simvastatin, and metformin (500 mg twice a day). Initial laboratory evaluation revealed leukocytosis (11,000/mm3) and severe renal failure (blood urea nitrogen 89 mg/dL; creatinine, 10.2 mg/dL), as well as a potassium level of 6.2 mmol/L, anion gap of 24, and glucose of 198 mg/dL. His lactate was 11 mmol/L with an arterial blood gas of pH 6.5 and CO2 0.05) and the angle between FNV and LTV (40.2 ± 9.7 vs 38.3 ± 9.6, P > 0.05) were not significantly different between groups. In conclusion, the lesser trochanteric retroversion is significantly increased in patients with psoas impingement compared with patients with asymptomatic hips. July 2015
Beyond Typhoid Mary: the sophistication of Salmonella typhi Charis Santini,* Curtis R, Mirkes, DO, and Cedric Spak, MD (*e-mail: santini@ medicine.tamhsc.edu)
As resistant strains of Salmonella typhi are increasing worldwide, a clinician in the United States may find it more difficult to treat typhoid fever in returning travelers. This case demonstrates the variability within which typhoid fever can present. A 20-yearold formerly healthy man presented with an 18-day history of intermittent fever and myalgia. The fever spiked to approximately 104°F in the late afternoons. Associated symptoms included chills, headache, weakness, nausea/vomiting, and occasional loose stools. The patient returned from Bangladesh 5 days prior to symptom onset. He denied contact with contaminated food, sick contacts, bloody diarrhea, constipation, or rashes. His laboratory results were significant for a white blood cell count of 4.7 × 109/L with 37% bands, thrombocytopenia (109 × 109/L), hyponatremia (129 mmol/L), and elevated transaminases (aspartate transaminase [AST] and alanine transaminase [ALT], 157 and 138 U/L). Cultures were obtained and the patient was placed empirically on vancomycin and piperacillin/tazobactam. Serum cultures were positive after 48 h for gram-negative rods. After 3 days of treatment, the patient’s symptoms did not improve. His liver transaminases continued to increase to a maximum AST/ALT of 301/245 U/L. Also, his hemoglobin and hematocrit levels dropped, with laboratory values consistent with intravascular hemolysis. The patient improved on ceftriaxone. Culture growth further revealed S. typhi group D with intermediate sensitivity to ciprofloxacin. He was discharged on oral antibiotics. This case of typhoid fever was not the straightforward picture of enteritis, bloody diarrhea/constipation, and salmon-colored macules. Once typhoid fever is diagnosed, a new difficulty arises with the changing resistance patterns; selecting appropriate antibiotic therapy is of the utmost importance. Fluoroquinolones for 7 to 10 days is the recommended treatment. However, in certain regions, including South Asia, fluoroquinolone resistance is becoming more prevalent. Therefore, in cases of known fluoroquinolone resistance, recommendations promote the use of a third-generation cephalosporin or azithromycin. Delaying appropriate therapy increases the risk of relapse and can result in a mortality rate of 10% to 20%. Acute enlargement and dysfunction of the exocrine glands of the head Avery Smith, MD,* and Alan Hise (*e-mail: [email protected])
We present a case of immunoglobulin G4-related disease (IgG4-RD), a recently recognized syndrome. Diagnosis generally requires elevated serum IgG4 levels, biopsy-proven elevation in IgG4, and a rapid response to steroid treatment. A 23-yearold Ethiopian man presented with 2 weeks of facial swelling of his upper eyelids, submandibular area, and cheeks. He endorsed visual impairment, nasal stuffiness, dry mouth/eyes, and odynophagia. Laboratory values were normal except for eosinophilia. Magnetic resonance imaging of the head demonstrated significant bilateral lacrimal gland swelling and buccal lymphadenopathy. The patient began to have a sensation that his throat was “closing up” so was given high-dose intravenous dexamethasone.
Abstracts from the First Annual Scholarly Day
323
Subsequently, he had dramatic improvement. A bilateral lacrimal gland biopsy showed many B and T lymphocytes mixed with eosinophils. The lymphocytes were prominently positive for IgG. Only 1% of the lymphocytes were positive for IgG4, but his serum IgG4 level was slightly elevated at 97 mg/dL. He was transitioned to oral prednisone. After completing the steroid taper, his facial features were normal, eosinophilia resolved, and he was asymptomatic. He has not had a relapse to date. Enlargement of the lacrimal and salivary glands, known as Mikulicz’s disease, was once considered a subtype of Sjögren syndrome. This disease has since been reclassified as falling within the IgG4-RD spectrum. This case had many features of IgG4-RD but did not meet strict diagnostic criteria. However, suspicion for IgG4-RD remains high based upon his presentation, response to treatment, and exclusion of other diagnoses. Antinuclear antibody and anti-Ro/SSA and anti-La/SSB antibodies were negative. The marked peripheral eosinophilia and prominent eosinophils in his lacrimal gland biopsy suggested an allergic etiology. However, serum IgE levels were normal. Thus, his peripheral and biopsy-proven eosinophilia was attributed to a reactive process secondary to the recruitment of T lymphocytes, a well-recognized feature of IgG4-RD. The patient’s dramatic response to dexamethasone and the eventual resolution of his dry eyes/mouth support IgG4-RD over Sjögren syndrome. Thus, it appears that intensive steroid treatment can markedly decrease peripheral and glandular IgG4 levels, and this may have accounted for his negative results. A rare case of recurrent focal myositis and an associated coagulopathy Avery Smith, MD,* and Carolyn Quan, MD (*e-mail: Avery.Smith@ baylorhealth.edu)
Focal myositis is a rare disorder with less than 50 cases reported to date. It typically presents as localized swelling, pain, and skin discoloration over a muscle group of an extremity. We present a case of recurring focal myositis complicated by an autoimmune coagulopathy. A 74-year-old African American man presented to the emergency department with a 3-day history of right calf swelling, dark discoloration, and pain, which started abruptly without inciting trauma. He denied fever, chills, or night sweats, but did note a similar presentation 2 months earlier in his left upper extremity. Based on magnetic resonance imaging, myositis was diagnosed, and the patient was treated with antibiotics despite negative cultures. He improved but then returned with the right calf pain. Laboratory results showed markedly positive antinuclear antibody, positive anti-centromere antibody, and an elevated erythrocyte sedimentation rate/C-reactive protein. Surgical muscle biopsy showed necrosis and muscle regeneration but no infection, dermatomyositis, polymyositis, or inclusion body myositis. The procedure was complicated by significant bleeding. Studies showed an isolated prolongation of the partial thromboplastin time, which led to the diagnosis of a factor VIII inhibitor.
324
Electromyography performed to evaluate for subclinical weakness had unremarkable results. However, his myositis recurred at the electromyography insertion sites. He was then treated with high-dose prednisone, rituximab, and cyclophosphamide. He improved without further bleeding or recurrence of his myositis. This case is unique because the patient experienced recurrent focal myositis as well as the development of a rare autoimmune-driven coagulopathy. Heffner’s theory regarding micro-trauma as an inciting factor was supported by this patient’s recurrent myositis following the electromyography study. Focal myositis is an atypical, rheumatologic syndrome that can mimic infections of other rheumatologic entities. It is presumably secondary to an autoimmune response against a muscular antigen, but the specific antibody remains unknown, with negative myositis panels, antistriated muscle antibody, and paraneoplastic panel. Delayed treatment for herpes zoster resulting in disabling plexopathy in the immunosuppressed patient Anwar Y. Zaman, MD,* Sridevi Mukkamala, MD, and Matt Bayazitoglu, MD (*e-mail: [email protected])
Here we report two cases of adults on chronic immunosuppressive therapy who were diagnosed with herpes zoster and treated with oral steroids alone. Both patients developed focal weakness in the affected limb. A 63-year-old man on chronic immunosuppression after liver transplantation presented to his primary care physician with a left lower extremity vesicular rash and pain. He was initially started on oral steroids and within 5 weeks developed left lower extremity weakness with foot drop. He subsequently was admitted to the hospital and started on antiviral treatment. The second patient was a 66-yearold man with rheumatoid arthritis on chronic immunosuppression who presented to his primary care physician with a right thigh vesicular rash and pain. He was started on oral steroids and 1 month later developed progressive right lower extremity weakness. Nerve conduction studies and electromyography were performed on the patients more than a month after onset of symptoms. Each patient had electrodiagnostic evidence of lumbosacral plexopathy with significant motor denervations that matched clinical findings. Lumbosacral plexopathy with motor involvement occurring in the context of herpes zoster is rare and underrecognized. The patients each developed severe weakness with minimal recovery at 2-month follow-up. The patients did not receive antiviral treatment at the time of initial rash presentation. The delay in antiviral treatment may have contributed to dissemination of the infection and extensive motor involvement. In conclusion, patients on chronic immunosuppressive therapy can be at higher risk of developing plexopathy or polyradiculopathy secondary to herpes zoster. In this patient population, early disease recognition and treatment with antiviral medication may help prevent plexopathy and subsequent disability.
Baylor University Medical Center Proceedings
Volume 28, Number 3
