Zucker School of Medicine at Hofstra/Northwell at Mather Hospital
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First Author: Aikta Rajput, MD Co-Author: Ernesto Caban, MD – Internal Medicine Resident, Northwell Health - Mather Hospital Co-Author: John Kennedy, MD, MSc – Pulmonologist/Intensivist, Stony Brook University Hospital Co-Author: Anish Desai, MBBS – Pulmonologist/Intensivist, Stony Brook University Hopsital
Introduction: Hypersensitivity pneumonitis-related interstitial lung disease (HP-ILD) is an immune-mediated lung condition caused by repeated inhalation of environmental antigens, leading to inflammation and, in some cases, fibrosis. It is categorized as fibrotic or non-fibrotic, with fibrotic HP-ILD linked to worse outcomes. Antigen avoidance remains key, but corticosteroids are often required when imaging or pathology shows inflammation. In chronic disease, steroid-sparing agents like mycophenolate mofetil (MMF) and azathioprine (AZA) may help preserve lung function, though their efficacy may be reduced in patients with telomere dysfunction. Rituximab has shown benefit in refractory disease, and lung transplant may be considered in advanced cases. Acute exacerbations can present with worsening dyspnea, cough, fever, and increased oxygen needs. While steroids are first-line, intravenous immunoglobulin (IVIg), with immunomodulatory properties, has shown benefit in fibrotic ILD flares.
Description: A 73-year-old man with biopsy-confirmed fibrotic HP-ILD and coronary artery disease presented with cough, dyspnea, and flu-like symptoms. He was initially treated for presumed pneumonia with antibiotics and IV methylprednisolone (60 mg daily). His condition worsened, requiring higher oxygen support. An acute HP-ILD exacerbation was diagnosed, and he was transferred to a step-down unit. Treatment was escalated to IV methylprednisolone (125 mg twice daily) and IVIg (5 g daily for three days). The patient improved, with decreased oxygen needs and symptom resolution. Infections were ruled out. He was discharged on oral prednisone (60 mg twice daily) with plans to assess telomere length and start MMF if telomeropathy was absent.
Discussion: IVIg may serve as a useful adjunct in managing acute HP-ILD exacerbations requiring high oxygen support. Its immunomodulatory actions complement therapies targeting T-cell (MMF, AZA) and B-cell (rituximab) pathways. Corticosteroids remain the foundation of treatment, but steroid-sparing agents offer long-term disease control, particularly in patients without telomeropathy. Though evidence for IVIg is limited, this case supports its potential role. Further research is needed to clarify its efficacy, dosing, and integration into long-term care plans.
