title: Predictors of granulomatous lymphocytic interstitial lung disease in common variable immunodeficiency.
abstract: A subset of patients with common variable immunodeficiency (CVID) develop granulomatous lymphocytic interstitial lung disease (GLILD), which is associated with early mortality.
  
  
  
  To determine a set of clinical and/or laboratory parameters that correlate with GLILD.
  
  
  
  A retrospective, nested case-control (patients with CVID diagnosed with GLILD compared with patients with CVID without a diagnosis of GLILD) medical record review was undertaken at Mayo Clinic, Rochester, MN. Network and univariate analysis was used to identify clinical and laboratory parameters at the time of diagnosis that are associated with GLILD.
  
  
  
  Twenty-six cases with radiologic evidence of GLILD were included in this study. Eighteen cases (69%) cases had coexistent splenomegaly with lower IgA levels (P = .04) compared with the controls. Patients with low IgA levels (<13 mg/dL) also had percentage expansion of low CD21 B cells (CD21low >5%) (P = .007). Univariate analysis revealed that splenomegaly (odds ratio [OR], 17.3; 95% confidence interval [CI], 3.9-74.5), history of immune thrombocytopenic purpura (ITP) or autoimmune hemolytic anemia (AIHA) (OR, 4.8; 95% CI, 1.1-20.2), low IgA level (OR, 3.6; 95% CI, 1.2-11.9), and percentage expansion of CD21low (OR, 5.8; 95% CI, 1.6-24.7) were independently associated with GLILD. Logistic regression analysis revealed that splenomegaly, history of ITP or AIHA, low IgA level, and percentage expansion of CD21low B cells are highly sensitive in predicting presence of GLILD (area under the receiver operating curve of 0.86).
  
  
  
  Presence of splenomegaly, history of ITP or AIHA, low serum IgA level, and percentage expansion of CD21low B cells may be useful to identify a group of patients at high risk for development of GLILD.
authors.affiliation: Mayo Medical School, Rochester, Minnesota.
authors.forename: Stella
authors.initials: S
authors.lastname: Hartono
authors.affiliation: Division of Allergic Diseases, Mayo Clinic, Rochester, Minnesota.
authors.forename: Megan S
authors.initials: MS
authors.lastname: Motosue
authors.affiliation: Department of Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, Minnesota.
authors.forename: Shakila
authors.initials: S
authors.lastname: Khan
authors.affiliation: Department of Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, Minnesota.
authors.forename: Vilmarie
authors.initials: V
authors.lastname: Rodriguez
authors.affiliation: Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, Minnesota.
authors.forename: Vivek N
authors.initials: VN
authors.lastname: Iyer
authors.affiliation: Division of Allergic Diseases, Mayo Clinic, Rochester, Minnesota.
authors.forename: Rohit
authors.initials: R
authors.lastname: Divekar
authors.affiliation: Division of Allergic Diseases, Mayo Clinic, Rochester, Minnesota; Department of Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, Minnesota. Electronic address: joshi.avni@mayo.edu.
authors.forename: Avni Y
authors.initials: AY
authors.lastname: Joshi
mesh_terms: D000465:Algorithms; D001402:B-Lymphocytes / Q000276:immunology / Q000378:metabolism / Q000473:pathology; D015415:Biomarkers; D016022:Case-Control Studies; D017074:Common Variable Immunodeficiency / Q000150:complications*; D005260:Female; D006099:Granuloma / Q000473:pathology*; D006801:Humans; D007132:Immunoglobulin Isotypes / Q000097:blood / Q000276:immunology; D007958:Leukocyte Count; D017563:Lung Diseases, Interstitial / Q000175:diagnosis* / Q000209:etiology*; D008297:Male; D010641:Phenotype; D012372:ROC Curve; D012129:Respiratory Function Tests; D012189:Retrospective Studies
chemical_list: D015415:Biomarkers; D007132:Immunoglobulin Isotypes