[CIS PIDD] [cis-pidd] Renal Disease and CVID

Fri Mar 25 03:30:52 EDT 2016

Hi Jason,

we have had one idiopathic CVID with granulomatous unilateral inflammation, controlled with CS.

The CD8 rich infiltrate really makes one think of intracellular infection or CD8+ LGL lymphoproliferation. Kidneys would not be a typical site for either but has been described  in immunocompromised.
Blood smear: LGL excess in blood? Hepatomegaly/biopsy? BM sample? TCR clonslity? In our cohort of CVIDs, about 2% / 3 patients have LGL-L.

And looking at her other results, I would exclude also a germline STAT3 GOF.

Hope this helps, ATB

Mikko

Oyl Mikko Seppänen
Harvinaissairauksien yksikkö (HAKE)

Head, Rare Disease Center,
Helsinki University Hospital (HUH)
FINLAND

phone +358 947180201
GSM +358 50 4279606
fax +358 9 47174703

CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>> kirjoitti 24.3.2016 kello 22.23:

All,
I have a complex patient that I would like to present.

This is a 24 year old white female with CVID.  She presented to me in 2012.  Prior to my seeing her she was followed by peds Hem/Onc and then adult Hem/Onc for ITP (now resolved).  Her treatment included IVIG and rituximab.  On my initial evaluation her main infection was recalcitrant sinus disease.  My initial work up showed her to be:
Agammaglobulinemic with undetectable IgG, IgA, and IgM
Titers:  No response to pneumovax, tetanus, or diphtheria
Current T and B cell numbers
B cell 1% and 30 cells/uL
CD3  96.7% and 2420 cells/uL
CD4  19.5% and 490 cells/uL
CD8 77.5% and 1940 cells/uL
CD4/CD8 0.3

CD27+ B cells < 1%.

T cell function normal at initial consult.

Comorbid conditions:
ITP (resolved)
Lymphoid hyperplasia
Hepatosplenomegaly
Granulomatous Skin Disease
FINAL PATHOLOGIC DIAGNOSIS
MICROSCOPIC EXAMINATION AND DIAGNOSIS

SKIN, RIGHT JAWLINE, BIOPSY:
Sparse dermatitis with immature granulomas..
Lung Disease (Considering further lung studies and GLILD)
CT OF THE CHEST, ABDOMEN AND PELVIS WITH INTRAVENOUS CONTRAST,
.
INDICATION: adenopathy
.
COMPARISON: CT abdomen and pelvis 1/10/2014
.
TECHNIQUE: After administration of intravenous contrast, axial images of the chest, abdomen and pelvis were obtained in the portal venous phase. Supplemental 2D reformatted images were generated and reviewed as needed.
.
CHEST:
. Chest wall/thoracic inlet: Within normal limits.
. Thyroid: Incompletely imaged 13 mm low-density lesion in the left thyroid lobe.
. Mediastinum/hila: Within normal limits.
. Heart/vessels: Within normal limits.
. Lungs: Multiple groundglass foci are present predominantly in the right middle and bilateral lower lobes. 9 mm right middle lobe nodule (series 4, image 58). Several additional groundglass nodules are present in the periphery of the right lower lobe. 12 mm groundglass nodule in the right lower lobe (image 76, series 4).
CONCLUSION:
No substantial change in appearance of upper abdominal ligament and retroperitoneal adenopathy.
Patchy enhancement of the kidneys, concerning for pyelonephritis. Correlation with urinalysis is recommended.
Several groundglass nodules are present in both lungs which may be infectious or inflammatory in etiology. Recommend continued attention on follow-up.

Current clinical issue:
                In 12/2015 she presented with fever and flank pain.  She was admitted, diagnosed and treated for  pyelonephritis.  At this time she was found to have a creatinine of 1.91 (her weight is only 46.7 kg).   She was thought to have acute renal failure, but unfortunately he elevated creatinine has persisted and increased to 2.1.  Nephrology became involved and she had a renal biopsy showing a CD3+ CD68+ diffuse infiltrate with near complete interstitial fibrosis(note:  The biopsy was very small and not optimal) . A laparoscopic renal  biopsy has been completed and shows: scattered and few B cells or plasma cells.  A predominant CD8 interstitial infiltrate (80%). The infiltrate is not represented of the entire kidney with the larger biopsy, but 1 of 15 glomeruli are fibrosed.
                An infectious work up is negative for: HIV, CMV, EBV, JC virus, BK virus, HSV and pending for a GI panel, TB gold, repeat CT of Chest, Ab, and Pelvis, and HHV-8.  Urine has always been negative.
                My search of the literature shows a paucity of CVID patients with renal involvement.  Most in fact are case reports.  This renal literature reports the use of corticosteroids for disease suppression for varying lengths of time and success.

  1.  Has anyone see this type of infiltrate in the kidneys?
  2.  What other comments or recommendations do you have?

Thank you in advance for your attention,

Jason

PS: For those who are interested, she has one health boy born in 2014.  She was treated and monitored on sub q IgG replacement throughout the pregnancy and the baby was follow for several months after birth.  He had normal IgG levels though out this period.

Jason W Caldwell DO FAAAAI
Associate Professor Internal Medicine & Pediatrics
Section of Pulmonary, Critical Care, Allergic and Immunological Diseases
Director of Allergy & Immunology
Program Director Allergy & Immunology Fellowship
Wake Forest School of Medicine
p 336.716.4843/f 336.716.7277
jcaldwel at wakehealth.edu<mailto:jcaldwel at wakehealth.edu> / WakeHealth.edu<http://wakehealth.edu>
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Jason W Caldwell DO FAAAAI
Associate Professor of Internal Medicine and Pediatrics
Section of Pulmonary, Critical Care, Allergic and Immunological Diseases
Director of Allergy/Immunology
Program Director of Allergy/Immunology Fellowship
Wake Forest University School of Medicine
Office: 336-716-5166
Administrative: 336-716-4843
Pager: 336-806-8330
jcaldwel at wakehealth.edu<mailto:jcaldwel at wfubmc.edu>
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