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

[dmvascon at usp.br]

Dear Dr. Gonzalez

We have some patients presenting severe granulomatous lesions in  
several organs. Despite good response to corticosteroids in some  
patients, other do not respond well and adverse effects are a  
significant drawback.
Initially we thought about using anti-TNF immunobiologicals (there are  
some reports of the use of infliximab: Ann Allergy Asthma Immunol.  
2005 Sep;95(3):293-300. Clin Exp Immunol. 2007 Nov;150(2):306-11).
After discussion of these manifestations with Dr. Charlotte  
Cunningham-Rundles she suggested the use of hydroxychloroquine. We are  
using these approach for some patients with severe granulomatous lung  
manifestations with good response and mild adverse effects. This  
approach could be interesting to spare steroids in your patient.

Best regards,

Dewton Vasconcelos
University of São Paulo



Citando Nacho Gonzalez <nachgonzalez at gmail.com>:


> 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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