Pulmonary Interstitial Disease in Ig Deficiency

An Official American Thoracic Society Clinical Practice Guideline: Classification, Evaluation, and Management of Childhood Interstitial Lung Disease in Infancy

“…All patients with DLD should be evaluated for immunodeficiency, because infections can cause DLD (116,128,(183)(184)(185)(186). Categorizing any detected immunodeficiency as primary (e.g., severe combined immunodeficiency) (187)(188)(189)(190), acquired (e.g., human immunodeficiency virus) (191), or due to immunosuppressive medication may help guide evaluation and treatment.…”

Cryptogenic organising pneumonia

“…Other disorders with associated organising pneumonia include: common variable hypogammaglobulinaemia and other immunoglobulin deficiencies [386][387][388]; polyarteritis nodosa [389]; Sweet syndrome [390][391][392]; polymyalgia rheumatica [393,394]; Behçet disease [395]; thyroid disease (including cancer, Basedow disease, thyroiditis, hypothyroidism) [396]; and sarcoidosis (with organising pneumonia at the periphery of granulomatous lesions) [397]. It was also reported after coronary artery bypass graft surgery [398] or in association with localised giant inflammatory polyposis of the caecum and distal ulcerative colitis [399].…”

Lymphocytic interstitial pneumonia and pulmonary embolism in a patient with tetralogy of Fallot and common variable immunodeficiency: is there any link?

“…It has been described in cases of hypogammaglobulinaemia, common variable immunodeficiency, monoclonal and polyclonal gammopathy, Sjögren’s syndrome and HIV infection, among others 1. Some patients have been successfully treated with prednisone and/or intravenous immunoglobulins, as previously reported 1 – 3…”

“…Extremities revealed distal cyanosis, but no clubbing or oedema. White blood cell count was 20 000/mm3 with 96% neutrophils and 3% lymphocytes and β-natriuretic peptide level was 256 pg/ml.…”