# Granulomatous Lymphocytic Interstitial Lung Disease Associated With Common Variable Immunodeficiency Presenting With Respiratory Failure

## TL;DR

A teenager with a rare immune disorder and lung disease rapidly improved after aggressive treatment for severe respiratory failure.

## Contribution

Highlights a rare case of acute respiratory failure in CVID-associated GLILD and the effectiveness of aggressive treatment.

## Key findings

- The patient showed rapid recovery with high-dose steroids and immunoglobulin therapy.
- Lung biopsies confirmed the diagnoses of CVID and GLILD.
- Early aggressive intervention improved outcomes in acute GLILD cases.

## Abstract

This case study presents a rare occurrence of acute respiratory failure in a 17-year-old male diagnosed with common variable immunodeficiency (CVID) and granulomatous lymphocytic interstitial lung disease (GLILD), which typically have a gradual onset. The patient initially exhibited nonspecific symptoms such as dry cough and fever but quickly progressed to severe respiratory failure despite conventional treatments. Imaging showed extensive lung abnormalities, and blood tests revealed significantly low immunoglobulin levels, indicating an underlying immunodeficiency. Treatment with high-dose steroids and immunoglobulin replacement therapy resulted in a rapid and remarkable recovery of lung function. Lung biopsies confirmed the dual diagnoses of CVID and GLILD, emphasizing the challenge of diagnosing and managing GLILD in CVID patients. This case underscores the importance of early and aggressive intervention in improving outcomes for GLILD patients with acute respiratory distress.