[PAGID] neuro+lung granulomata CVID how long steroids?

[Nacho Gonzalez]

Dear all, I need help with this case: 4-1/2 year old Romarian girl. In Feb
'08 mesencephalic mass with hemiparesis + hypogammaglobulinemia with absent
response to vaccines and nearly absent switched memory B cells with elevated
CD21low cells. Oct´08 developed periportal hepatitis (biopsied) and
interstial lymphoid pneumonitis (Lung biopsy) and mediastinal biopsy
granulomata were observed. Since Feb´08 to oct´08 was treated with
antituberculous drugs for suspected TB (no mycobacteria isolated). The brain
mass has disappeared after steroid treatment and she has recovered
completely from her neurological disabilities (intense tremor and
hemiparesis)
We have assumed the patient has the granulomatous subset of the CVID.
Now the patient is ok. These worsenings have ocurred at the same time
steroids were tappered off. This is why she receives 0.25 mg/kg daily.
The Densitometry shows osteopenia. (Zscore -2.6 in L1) so she started with
Calcium and vitamin D.

So does anybody know how long should I treat this patient with steroids? the
dose?

Many thanks,

Nacho Gonzalez. Immunodeficiencies Division. Hospital 12 octubre. Madrid.
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