50 m year old female  with combined variable immunodeficiency syndrome

• 49 y.o. female
  • hx of
    • sinusitis, pneumonia, and bronchitis. bronchiectasis in setting of CVID.She was diagnosed with low IgG around 19 years ago and started on IgG replacement in 4 years later, the transitioned
  • Diagnosed with CVID due to low IgA and, later, low IgM.

3 years ago

Pulmonary Nodules and lymph – adenopathy splenomegaly

• CT chest from 3 years ago
  • Interval increase in the size and number of numerous pulmonary nodules with
  • basal predominance compared to the prior
  • consistent with granulomatous lymphocytic interstitial lung disease
  • ? underlying/superimposed infection
  • stable bronchiectasis and consolidation in the medial right middle lobe and lingula.
  • Stable bilateral hilar lymphadenopathy.

 

1 year ago

Unchanged Symptoms

• Interval increase in size, confluence, and number of irregular widespread pulmonary nodules.
  • suggestive of progressive granulomatous lymphocytic interstitial lung disease.
  • Stable right middle lobe and lingular peribronchial consolidations with bronchiectasis.
  • Stable mediastinal and hilar adenopathy.

 

 

Current

•  lack of fever or acute deterioration,
  • growth of the only normal upper respiratory flora on the recent sputum culture
  • Sputum culture negative
• Imaging shows progressive
  • granulomatous interstitial lung disease (GL ILD) and
  • lymphocytic  interstitial lung disease
    • associated with CVI D
      • bronchiectasis