[CIS PIDD] [cis-pidd] Difficult patient

[Church, Joseph]

Colleagues:



I saw a 16yo boy with recurrent brain lesions and miliary lung nodules; he may have "CVID."

  *   2004: headache + vomiting.  Brain CT and f/u MRIs demonstrated enhancing mass 3x2.5.2.5cm right occipital lobe.  Pt treated with steroids for symptoms; ~3days later brain biopsy demonstrated T-cell infiltrative process without granulomas or clonal expansion .  Further steroid therapy was associated with disappearance of lesion.
  *   9/2006: headaches.  MRI showed 3+ cm area of decreased attenuation right cerebellum.  Bx NOT repeated.  Neurologist considered process to be same as lesion of 2004. Rx'd with steroids.
  *   11/2006:  Incidental lung lesions noted at scanning.  CT then demonstrated multiple, diffuse nodules 1 to 6 mm in size throughout lungs.  Biopsy showed non-caseating granulomas.  Improved with steroids given for brain.  (Also, incidental horseshoe kidney noted.)
  *   11/2006:  IgG 334, IgA 44, IgM 51, IgE 339.
  *   12/2006:  Poor antibody responses to Pneumovax and IVIG started.  Methotrexate given for brief (?) time.
  *   1/2010:  Brain lesions progressed; steroid increased, then tapered
  *   4/2012:  Brain lesions again progressed; stereoids increased, now tapering
  *   Current:  Continues to require systemic steroids to control recurrences of brain lesions; osteopenia, cushinoid, acne, 4+ striae.

Extensive evaluations for infectious and autoimmune etiologies have not documented a unifying diagnosis.  I have a notebook (literally) of details.



Given the very long term steroid therapy, I would guess that if the process were infectious he would not be alive.



It is tempting to relate the brain and lung lesions to the granulomatous process associate with CVID.  EXCEPT the brain lesions, at the time of biopsy in 2004, were not granulomatous.  Does this matter?



Is the brain process consistent with sarcoidosis?



Regardless, I think he needs more than steroids for the process.



Any thoughts would be most welcome.



Joe Church

Children's Hospital Los Angeles











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