[PAGID] FW: ? Granulomatous Angiitis

[Kate Sullivan]

Gosh- I hope it wasn't me who saw her!

I would give some thought to the weird RAG deficiencies described in  
this article.

http://content.nejm.org/cgi/content/full/358/19/2030


Kate
On Sep 29, 2009, at 5:53 PM, Vogler, Larry B wrote:


>

> I would very much appreciate any help that you may provide in the  

> diagnosis and management of this patient.

>

> The patient is a 10 year old East Indian girl with a normal birth.   

> She had some episodes of pharyngitis and gastroenteritis after age 4  

> months and was hospitalized at age 10 months at Children’s  

> Healthcare of Atlanta-Egleston with well-confirmed adenoviral  

> hepatitis. At 19 months in 12/00 she had an episode of IgG auto- 

> immune hemolytic anemia. In 12/03 she had pneumonia and in 3/05  

> bronchitis and otitis media. She had an ear drum perforation in 5/06  

> and was diagnosed with asthma in 4/08. In 11/08 she was noted to  

> have growth retardation and nail clubbing.  A chest CT showed  

> extensive bronchiectasis. In 1/09 she developed swelling of her left  

> elbow and left ankle associated with tender subcutaneous nodules. An  

> MRI showed lytic lesions in the distal humerus and increased marrow  

> signal in the proximal left radius.

> She has had high ESRs (118) and the following additional labs: WBC  

> 4.0: 69% segs, 17% lymphs, 7% monos, 7% eos, Hgb 9.3, platelets 234.  

> IgG 2280, IgA 60, IgM 92, IgE <2, CH50 51. CD4 lymphocytes 27%  

> (absolute ct.  295), CD8 9% (101), CD 19 14% (164), CD 16/56 37%  

> (420). Good responses to PHA and Con A in vitro, normal NK killing  

> in vitro and normal neutrophil oxidative burst.  ANCA was negative,  

> ANA + 1:160, ACE 20 (nl).  TB skin test negative, and negative  

> routine, fungal and AFB cultures and serologies for Brucella,  

> Bartonella, Histoplasma and HIV.

> She has been evaluated at MCG, CHOP and Emory. A biopsy of an elbow  

> soft tissue lesion on 5/11/09 was interpreted by our pathologist as  

> showing necrotizing angiocentric granulomatous inflammation with  

> granulomatous angiitis of small to medium-sized arterioles. A recent  

> bronchoscopy  showed signs of chronic bronchial inflammation but no  

> organisms. An abdominal ultrasound showed modest splenomgaly with  

> normal echogenicity and a brain MRI was normal.  A recent  

> ophthalmology exam revealed no uveitis or retinal lesions.  Attached  

> are photos of the skin lesions and histopathology.

> We believe that she has necrotizing sarcoid granulomatosis as  

> described in the following Pub Med articles: PMID 17377369,  

> 12823717, 12784179, 12687600, 10232451, 8092646.  However, this is  

> not certain and we are reluctant to immunosuppress this young girl  

> with low lymphocyte numbers if other diagnoses are likely.

>

> Thanks for your thoughts.

> Larry

>

>

> Larry B. Vogler, M.D.

> Director, Division of Rheumatology and Immunology

> Department of Pediatrics

> Emory University

> 2015 Uppergate Drive

> Atlanta, GA 30322

> phone: 404-778-2400

> fax: 404-727-3757

>

>

>

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> <NSG-skin1.jpg><NSG- 

> skin3.jpg><09-07-08-1-3c.jpg><09-07-08-1-5c.jpg><09-07-08-1-12c.jpg>


Kathleen Sullivan MD PhD
Professor of Pediatrics
Chief, Division of Allergy Immunology
The Children's Hospital of Philadelphia
(p) 215-590-1697
(f) 267-426-0363




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