- Email: [email protected]
Current Asthma Prevalence in Working Adults—United States, 2009–2014
74
An Uncommon Condition in a Patient with Common Variable Immune Deficiency
Desha Jordan, MD, FAAP, Kelly Homlar, MD, Carolyn Lovell, MS, MAT, CGC, and Betty B. Wray, MD, FAAAAI; Medical College of Georgia at Augusta University, Augusta, GA. RATIONALE: There is not a published case report describing a common variable immunodeficiency (CVID) patient presenting with pigmented villonodular synovitis (PVNS). Commonly, PVNS presents in the knee or hip. Presentation with ankle involvement is much rarer, with only a few such cases reported. Progression to bilateral ankle involvement appears to have not been previously documented. METHODS: 27 year old female with CVID and clinical features of McCune-Albright Syndrome developed PVNS with bilateral ankle involvement. Physical examination showed cafe au lait spots, short stature and round facies. Fibrous dysplasia of the skull and long bones was notable on imaging. We explored the possibility of an immunologic connection between these disease states through a review of literature, laboratory results and imaging studies. RESULTS: Routine karyotype and genetic testing of the GNAS locus were normal. Two chromosomal microarray analyses have showed a 743.8 kb interstitial gain at 12p13.33 gene locus. PHA lymphocyte stimulation testing was normal. B and T cell counts were normal at 500/mcL and 356/ mcL respectively. NK cell count was low at 60/mcL with normal NK cell function. Serum immunoglobulins showed low IgA of 32 mg/dL, normal IgM of 43 mg/dL and IgG of 992 mg/dL on weekly subcutaneous immunoglobulin. CONCLUSIONS: A defect at the 12p13.33 locus may cause the phenotype of CVID and McCune-Albright syndrome, with PVNS.
75
Good's Syndrome: A Rare Adult-Onset Combined Immunodeficiency
Gargi Patel, Priyanka Batchu, and Amay Parikh; Rutgers Robert Wood Johnson, New Brunswick, NJ. RATIONALE: Good’s Syndrome is a rare combined immunodeficiency seen in patients with thymoma and typically presents in fourth or fifth decade of life. We present a case of an opportunistic infection leading to a diagnosis of Good’s syndrome. METHODS: A retrospective chart review of a 62 year old man with a history of thymoma and recurrent pneumonias for past six months who presented to the hospital with multifocal pneumonia. RESULTS: Patient’s hospital course was complicated by pancytopenia and severe sepsis secondary to multidrug-resistant Klebsiella bacteremia and CMV viremia. After a prolonged hospital course with bloodstream infection with opportunistic organisms, immunologic workup was pursued which revealed low immunoglobulins, low T- Helper cells, reduced CD4/ CD8 ratio, and no B cells. Immune work up and the history of thymoma were consistent with the diagnosis of Good’s Syndrome. Shortly after diagnosis, patient died from severe sepsis despite IVIG therapy. CONCLUSIONS: Good’s syndrome is diagnosed in patients with thymoma with hypogammaglobulinemia, defective T-cell mediated immunity, and low or absent B cells. Good’s syndrome should be suspected in all adults who presents with recurrent infections from encapsulated organisms or opportunistic organisms concerning for adult onset immunodeficiency. Furthermore, immune workup should be performed in all patients with thymoma who present with sinopulmonary infections in order to start therapy with IVIG reduce risk of more severe infections.
76
Bacterial meningitis in pediatric patient with C2 deficiency
Ryan D. Buckley, MD1, Snehal Patel, DO2, and Michael O. Daines, MD, FAAAAI3; 1Banner University Medical Center, Department of Internal Medicine, Tucson, AZ, 2Banner University Medical Center, Division of
Pulmonary, Allergy, Critical Care and Sleep Medicine, Tucson, AZ, 3University of Arizona, Tucson, AZ. RATIONALE: Homozygous C2 deficiency occurs in approximately 1 in 20,000 patients. Most patients with C2 deficiency remain asymptomatic; however, it is estimated 40% develop autoimmune diseases and 50% develop recurrent infections. It is associated with IgG deficiency and pyogenic infections with encapsulated bacteria - including Streptococcus pneumoniae, Haemophilus influenzae and Neisseria meningitides. Currently, there are no specific treatment guidelines for complement deficiencies. Here we present a case of a patient with C2 deficiency with a severe complication and our treatment of the disease. METHODS: Immunoglobulin measurement performed by Sunquest Information Systems. Pneumococcal antibody measurement performed by Focus Diagnostics, Inc. RESULTS: Ten-year-old Caucasian male with history of C2 complement deficiency (previously on subcutaneous Ig therapy) presented with fever and altered mental status. LP demonstrated milky CSF with significant pleocytosis (neutrophil predominant) with elevated protein and low glucose - concerning for bacterial meningitis. He was started on empiric Vancomycin and Ceftriaxone at meningitic dosing. CSF and blood cultures remained negative. HSV PCR and Coccidioides immitis titers were negative. MRI brain revealed leptomeningeal enhancement. IgG on admission was 707mg/dL. Streptococcus pneumoniae antibody 14 serotypes within normal range. Patient clinically improved and completed 14 day course of Ceftriaxone. He received one dose of IVIg at 400mg/kg prior to discharge and resumed weekly subcutaneous immunoglobulin therapy thereafter. CONCLUSIONS: This case outlines the disease presentation of C2 deficiency and treatment options with empiric antibiotics and IVIg.
77
Current Asthma Prevalence in Working Adults—United States, 2009–2014
Jacek M. Mazurek, MD, MS, PhD, and Girija Syamlal, MBBS, MPH; National Institute for Occupational Safety and Health, Centers for Disease Control and Prevention, Morgantown, WV. RATIONALE: Work-related asthma includes new-onset asthma caused by factors related to work and preexisting or concurrent asthma worsened by factors related to work. As much as 51% of adult asthma may be related to work. METHODS: To estimate the current asthma prevalence among U.S. workers, the 2009–2014 National Health Interview Survey data for _18 years were analyzed. Respondents currently employed adults aged > with current asthma were those who have ever been told by a doctor or other health professional that they had asthma and still have asthma. Currently employed adults were those who reported being employed during the week prior to the interview; those on layoff or retired were excluded. Adjusted prevalence odds ratios (PORs) and confidence intervals were calculated. RESULTS: Among an estimated 141 million employed adults, the current asthma prevalence was 6.5% (95%CI, 6.3 to 6.7%). Of the 21 industries, the highest asthma prevalence was in the health care and social assistance (8.8%). Of the 23 occupations, the highest asthma prevalence was in healthcare support (9.2%). The adjusted asthma PORs were significantly elevated among workers in the health care and social assistance (POR51.17) and education services (1.12) industries, and in the education, training, and library (1.17) and protective service (1.30) occupations. CONCLUSIONS: As many as 4.7 million working adults may have asthma related to work. Health care providers should ask workers with new or worsening asthma for occupational exposures and be alert to potential associations between workplace exposures and asthma symptoms.
SATURDAY
Abstracts AB23
J ALLERGY CLIN IMMUNOL VOLUME 139, NUMBER 2
An Uncommon Condition in a Patient with Common Variable Immune Deficiency
Desha Jordan, MD, FAAP, Kelly Homlar, MD, Carolyn Lovell, MS, MAT, CGC, and Betty B. Wray, MD, FAAAAI; Medical College of Georgia at Augusta University, Augusta, GA. RATIONALE: There is not a published case report describing a common variable immunodeficiency (CVID) patient presenting with pigmented villonodular synovitis (PVNS). Commonly, PVNS presents in the knee or hip. Presentation with ankle involvement is much rarer, with only a few such cases reported. Progression to bilateral ankle involvement appears to have not been previously documented. METHODS: 27 year old female with CVID and clinical features of McCune-Albright Syndrome developed PVNS with bilateral ankle involvement. Physical examination showed cafe au lait spots, short stature and round facies. Fibrous dysplasia of the skull and long bones was notable on imaging. We explored the possibility of an immunologic connection between these disease states through a review of literature, laboratory results and imaging studies. RESULTS: Routine karyotype and genetic testing of the GNAS locus were normal. Two chromosomal microarray analyses have showed a 743.8 kb interstitial gain at 12p13.33 gene locus. PHA lymphocyte stimulation testing was normal. B and T cell counts were normal at 500/mcL and 356/ mcL respectively. NK cell count was low at 60/mcL with normal NK cell function. Serum immunoglobulins showed low IgA of 32 mg/dL, normal IgM of 43 mg/dL and IgG of 992 mg/dL on weekly subcutaneous immunoglobulin. CONCLUSIONS: A defect at the 12p13.33 locus may cause the phenotype of CVID and McCune-Albright syndrome, with PVNS.
75
Good's Syndrome: A Rare Adult-Onset Combined Immunodeficiency
Gargi Patel, Priyanka Batchu, and Amay Parikh; Rutgers Robert Wood Johnson, New Brunswick, NJ. RATIONALE: Good’s Syndrome is a rare combined immunodeficiency seen in patients with thymoma and typically presents in fourth or fifth decade of life. We present a case of an opportunistic infection leading to a diagnosis of Good’s syndrome. METHODS: A retrospective chart review of a 62 year old man with a history of thymoma and recurrent pneumonias for past six months who presented to the hospital with multifocal pneumonia. RESULTS: Patient’s hospital course was complicated by pancytopenia and severe sepsis secondary to multidrug-resistant Klebsiella bacteremia and CMV viremia. After a prolonged hospital course with bloodstream infection with opportunistic organisms, immunologic workup was pursued which revealed low immunoglobulins, low T- Helper cells, reduced CD4/ CD8 ratio, and no B cells. Immune work up and the history of thymoma were consistent with the diagnosis of Good’s Syndrome. Shortly after diagnosis, patient died from severe sepsis despite IVIG therapy. CONCLUSIONS: Good’s syndrome is diagnosed in patients with thymoma with hypogammaglobulinemia, defective T-cell mediated immunity, and low or absent B cells. Good’s syndrome should be suspected in all adults who presents with recurrent infections from encapsulated organisms or opportunistic organisms concerning for adult onset immunodeficiency. Furthermore, immune workup should be performed in all patients with thymoma who present with sinopulmonary infections in order to start therapy with IVIG reduce risk of more severe infections.
76
Bacterial meningitis in pediatric patient with C2 deficiency
Ryan D. Buckley, MD1, Snehal Patel, DO2, and Michael O. Daines, MD, FAAAAI3; 1Banner University Medical Center, Department of Internal Medicine, Tucson, AZ, 2Banner University Medical Center, Division of
Pulmonary, Allergy, Critical Care and Sleep Medicine, Tucson, AZ, 3University of Arizona, Tucson, AZ. RATIONALE: Homozygous C2 deficiency occurs in approximately 1 in 20,000 patients. Most patients with C2 deficiency remain asymptomatic; however, it is estimated 40% develop autoimmune diseases and 50% develop recurrent infections. It is associated with IgG deficiency and pyogenic infections with encapsulated bacteria - including Streptococcus pneumoniae, Haemophilus influenzae and Neisseria meningitides. Currently, there are no specific treatment guidelines for complement deficiencies. Here we present a case of a patient with C2 deficiency with a severe complication and our treatment of the disease. METHODS: Immunoglobulin measurement performed by Sunquest Information Systems. Pneumococcal antibody measurement performed by Focus Diagnostics, Inc. RESULTS: Ten-year-old Caucasian male with history of C2 complement deficiency (previously on subcutaneous Ig therapy) presented with fever and altered mental status. LP demonstrated milky CSF with significant pleocytosis (neutrophil predominant) with elevated protein and low glucose - concerning for bacterial meningitis. He was started on empiric Vancomycin and Ceftriaxone at meningitic dosing. CSF and blood cultures remained negative. HSV PCR and Coccidioides immitis titers were negative. MRI brain revealed leptomeningeal enhancement. IgG on admission was 707mg/dL. Streptococcus pneumoniae antibody 14 serotypes within normal range. Patient clinically improved and completed 14 day course of Ceftriaxone. He received one dose of IVIg at 400mg/kg prior to discharge and resumed weekly subcutaneous immunoglobulin therapy thereafter. CONCLUSIONS: This case outlines the disease presentation of C2 deficiency and treatment options with empiric antibiotics and IVIg.
77
Current Asthma Prevalence in Working Adults—United States, 2009–2014
Jacek M. Mazurek, MD, MS, PhD, and Girija Syamlal, MBBS, MPH; National Institute for Occupational Safety and Health, Centers for Disease Control and Prevention, Morgantown, WV. RATIONALE: Work-related asthma includes new-onset asthma caused by factors related to work and preexisting or concurrent asthma worsened by factors related to work. As much as 51% of adult asthma may be related to work. METHODS: To estimate the current asthma prevalence among U.S. workers, the 2009–2014 National Health Interview Survey data for _18 years were analyzed. Respondents currently employed adults aged > with current asthma were those who have ever been told by a doctor or other health professional that they had asthma and still have asthma. Currently employed adults were those who reported being employed during the week prior to the interview; those on layoff or retired were excluded. Adjusted prevalence odds ratios (PORs) and confidence intervals were calculated. RESULTS: Among an estimated 141 million employed adults, the current asthma prevalence was 6.5% (95%CI, 6.3 to 6.7%). Of the 21 industries, the highest asthma prevalence was in the health care and social assistance (8.8%). Of the 23 occupations, the highest asthma prevalence was in healthcare support (9.2%). The adjusted asthma PORs were significantly elevated among workers in the health care and social assistance (POR51.17) and education services (1.12) industries, and in the education, training, and library (1.17) and protective service (1.30) occupations. CONCLUSIONS: As many as 4.7 million working adults may have asthma related to work. Health care providers should ask workers with new or worsening asthma for occupational exposures and be alert to potential associations between workplace exposures and asthma symptoms.
SATURDAY
Abstracts AB23
J ALLERGY CLIN IMMUNOL VOLUME 139, NUMBER 2