title: Granulomatous-lymphocytic interstitial lung disease and recurrent sinopulmonary infections in a patient with Good's syndrome.
abstract: Good's syndrome is a rare primary immunodeficiency associated with adult thymoma. Complications are mainly autoimmune manifestations and recurrent infections with encapsulated bacteria. Only one possible case of combined granulomatous-lymphocytic interstitial lung disease (GL-ILD) and Good's syndrome have been described earlier, but the patient died at the time of diagnosis. This is the first case of GL-ILD in Good's syndrome with a successful outcome. We present a case of a 43-year-old man with GL-ILD, who suffered from recurrent infections of Haemophilus influenzae and Pneumocystis jirovecii, with 8-year follow-up. After a thymectomy, he was diagnosed with Good's syndrome and GL-ILD. He was treated with prophylactic pivampicillin, quinolones and cephalosporins for his recurrent P. jirovecii and H. influenzae infections, an approach that proved unsuccessful due to resistance, with relapse after cessation. He was stabilised with oral diaminodiphenyl-sulfone for P. jirovecii and colistimethate-sodium inhalations for H. influenzae, which is a new approach to prophylactic treatment.
authors.affiliation: Department of Pulmonary Medicine, Aarhus University Hospital, Aarhus, Denmark.
authors.forename: Mads Lynge
authors.initials: ML
authors.lastname: Jensen
authors.affiliation: Department of Pulmonary Medicine, Aarhus University Hospital, Aarhus, Denmark.
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Bendstrup
authors.affiliation: Department of Pulmonary Medicine, Aarhus University Hospital, Aarhus, Denmark.
authors.forename: Ole
authors.initials: O
authors.lastname: Hilberg
mesh_terms: D000328:Adult; D000361:Agammaglobulinemia / Q000473:pathology; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D002511:Cephalosporins / Q000627:therapeutic use; D006099:Granuloma / Q000382:microbiology* / Q000473:pathology; D006192:Haemophilus Infections / Q000382:microbiology / Q000473:pathology*; D006193:Haemophilus influenzae / Q000302:isolation & purification; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000382:microbiology / Q000473:pathology; D017563:Lung Diseases, Interstitial / Q000188:drug therapy / Q000382:microbiology*; D008297:Male; D009375:Neoplasms, Glandular and Epithelial / Q000473:pathology; D010917:Pivampicillin / Q000627:therapeutic use; D016720:Pneumocystis Infections / Q000382:microbiology / Q000473:pathology; D045363:Pneumocystis carinii / Q000302:isolation & purification; D015363:Quinolones / Q000627:therapeutic use; D035583:Rare Diseases; D013945:Thymoma / Q000473:pathology; D013953:Thymus Neoplasms / Q000473:pathology
chemical_list: D000900:Anti-Bacterial Agents; D002511:Cephalosporins; D015363:Quinolones; D010917:Pivampicillin