[CIS PIDD] [cis-pidd] Difficult patient

[dmvascon at usp.br]

Hi Joe, good afternoon 

I have seen two patients with CNS manifestations in the context of CVID. 

The first of them is a young female sent to evaluation of ALPS: she had Evans syndrome and high double-negative T cells; she presented low levels of all Ig isotypes and improved after IVIg replacement (500 mg/kg/month). One year later she developed hemiparesthesia and hemiparesis diagnosed as CNS vasculitis by brain biopsy. After high dose steroids and IVIg for 5 months she improved and is now alive and well , without relapse of CNS disease. 

The second one was previously diagnosed as CVID and started to present seizures and localization signs, diagnosed as ADEM. She was treated as for herpesvirus and immunosuppressed improving for approximately one year and later started to present neurodegenerative features evolving to death. 

There are some cases of "sarcoid" manifestations of CVID in CNS. 
In some cases of "sarcoid" manifestations in the lungs and lymphnodes I tried to use hydroxychloroquine (400 mg/day), as suggested by Charlotte Cunningham-Rundles, with good responses in three cases. 

Best regards, 

Dewton 

Dewton de Moraes Vasconcelos 
University of São Paulo School of Medicine 

----- Mensagem original -----


> De: "Joseph Church" <JChurch at chla.usc.edu>

> Para: "CIS-PIDD" <cis-pidd at lists.clinimmsoc.org>

> Enviadas: Segunda-feira, 26 de Novembro de 2012 13:11:45

> Assunto: [cis-pidd] Difficult patient



> 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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