[CIS PIDD] [cis-pidd] Puzzling Case

[Naides, Stanley J]

The key question before taking up such a challenge is what criteria will
be used to determine that someone has "proven" ANA negative lupus?

What ANA method would be acceptable?

Stan 


Stanley J. Naides, M.D., F.A.C.P., F.A.C.R. 
Medical Director, Immunology R&D | Interim Scientific Director,
Immunology R&D
Quest Diagnostics Nichols Institute | Immunology | 33608 Ortega Highway

| San Juan Capistrano, CA 92675 USA | phone +1.949.728.4578 | fax

+1.949.728.7852 | Stanley.J.Naides at QuestDiagnostics.com |
www.NicholsInstitute.com

-----Original Message-----
From: Verbsky, James [mailto:jverbsky at mcw.edu] 
Sent: Wednesday, June 19, 2013 11:36 AM
To: CIS-PIDD
Subject: RE:[cis-pidd] Puzzling Case

Joe

I took the liberty of running your case by Dr Atkinson.  His thoughts
were this would be consistent with anti-C1q antibodies and HUVS.  I have
seen it in a young boy but much older than your patient.  His other
thought was cryoglobulins and SLE (whether complement deficiency such as
C3 or not).  He did offer a free steak dinner at one of the best
restaurants in St Louis for anyone with a PROVEN ANA negative lupus.  He
has had this bet for years...I dont think he has ever lost it.

Best

James
James Verbsky MD/PhD
Associate Professor of Pediatrics and Microbiology and Molecular
Genetics Medical College of Wisconsin Milwaukee, WI  53226
________________________________________
From: Church, Joseph [JChurch at chla.usc.edu]
Sent: Tuesday, June 18, 2013 5:53 PM
To: CIS-PIDD
Subject: [cis-pidd] Puzzling Case

Colleagues:

Rheumatology referred a now 6 month infant girl who since 5 weeks of age
has had a persistent, shifting, generalized rash characterized by
irregular reddish-brown plaques (see attached picture) that are
completely non-pruritic.  A biopsy showed "urticarial vasculitis."  A
brief course of prednisone resulted in transient complete clearing of
the rash.  The infant is growing and developing normally; she has had no
serious infections; her exam is normal except for the rash; there are no
joint findings.  Labs:

CH50 and AH50 were '0' (repeatedly).
All classical pathway component tested were low.
RF, CIC, anti-C1q all positive
ANA, anti-dsDNA, SSA and SSB all negative (mom also).
UA normal
Extensive search for infectious agents has been unrevealing.
CBC shows neutropenia with positive anti-neutrophil antibody, and
anemia-Coombs pending.

Other than neonatal lupus any thoughts regarding diagnostic
interventions and possible therapy (?IVIG).

Joe Church
Children's Hospital Los Angeles












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