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

[CIS-PIDD]

Hi.

We have a quite similar patient.

39 year old female

Initial diagnosis in 2011 of lung, liver and cutaneous sarcoidosis
Presented to me in 2015 for refractory sinus infections
Agammaglobulinemic ( a posteriori, she was already agamma in 2011 )
Absent vaccin response

Phenotype
Total lymphocyte count 1200
B cell 4.5 % CD27+ 24%
CD3 77 %
CD4 32 %
CD8 44 %
Normal TReg


A couple of months after starting IVIG, her creatinin level rised up to 1.5 without obvious reason.


Renal biopsy pathological findings :
Granulomatous tubulo-interstitial nephritis with predominant lymphocytic infiltrate ( CD8 staining not done for technical reasons )
2 sclerosed glomeruli out of 22, no fibrosis


We planned to use combination of rituximab + MMF + prednisone
Corticosteroids were refused by the patient as she suffered from severe side effects from them in the past.
MMF was started alone, as rituximab insurance coverage was denied.


Her creatinin level completely normalized within 2 months with MMF only.
Her reponse has been sustained for 6 months now.

Although not as severe as your patient, I hope our experience will help in your reflection

Best regards

Hugo Chapdelaine
Professeur adjoint de clinique
Service d'immunologie clinique et allergie
Centre hospitalier de l'Université de Montréal


Le 24/03/16 à 16:23, CIS-PIDD  <cis-pidd at lists.clinimmsoc.org> a écrit :
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> All,
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> I have a complex patient that I would like to present.
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> 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:
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> Agammaglobulinemic with undetectable IgG, IgA, and IgM
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> Titers: No response to pneumovax, tetanus, or diphtheria
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> Current T and B cell numbers
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> B cell 1% and 30 cells/uL
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> CD3 96.7% and 2420 cells/uL
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> CD4 19.5% and 490 cells/uL
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> CD8 77.5% and 1940 cells/uL
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> CD4/CD8 0.3
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> CD27+ B cells < 1%.
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> T cell function normal at initial consult.
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> Comorbid conditions:
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> ITP (resolved)
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> Lymphoid hyperplasia 
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> Hepatosplenomegaly
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> Granulomatous Skin Disease
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>  FINAL PATHOLOGIC DIAGNOSIS
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> MICROSCOPIC EXAMINATION AND DIAGNOSIS
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> SKIN, RIGHT JAWLINE, BIOPSY:
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> Sparse dermatitis with immature granulomas..
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> Lung Disease (Considering further lung studies and GLILD)
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> CT OF THE CHEST, ABDOMEN AND PELVIS WITH INTRAVENOUS CONTRAST,
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> INDICATION: adenopathy
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> COMPARISON: CT abdomen and pelvis 1/10/2014
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> 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.
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> CHEST:
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> . Chest wall/thoracic inlet: Within normal limits.
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> . Thyroid: Incompletely imaged 13 mm low-density lesion in the left thyroid lobe.
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> . Mediastinum/hila: Within normal limits.
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> . Heart/vessels: Within normal limits.
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> . 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).
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> CONCLUSION:
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> No substantial change in appearance of upper abdominal ligament and retroperitoneal adenopathy.
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> Patchy enhancement of the kidneys, concerning for pyelonephritis. Correlation with urinalysis is recommended.
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> Several groundglass nodules are present in both lungs which may be infectious or inflammatory in etiology. Recommend continued attention on follow-up.
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> Current clinical issue:
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>  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. 
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>  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.
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>  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. 
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>  	Has anyone see this type of infiltrate in the kidneys?
> 	What other comments or recommendations do you have?
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> Thank you in advance for your attention,
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> Jason
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> 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.
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> Jason W Caldwell DO FAAAAI
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> Associate Professor Internal Medicine & Pediatrics
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> Section of Pulmonary, Critical Care, Allergic and Immunological Diseases
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> Director of Allergy & Immunology
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> Program Director Allergy & Immunology Fellowship
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> Wake Forest School of Medicine
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> p 336.716.4843/f 336.716.7277
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> jcaldwel at wakehealth.edu / WakeHealth.edu
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> Jason W Caldwell DO FAAAAI
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> Associate Professor of Internal Medicine and Pediatrics
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> Section of Pulmonary, Critical Care, Allergic and Immunological Diseases
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> Director of Allergy/Immunology
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> Program Director of Allergy/Immunology Fellowship
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> Wake Forest University School of Medicine
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> Office: 336-716-5166
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> Administrative: 336-716-4843
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> Pager: 336-806-8330
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> jcaldwel at wakehealth.edu <jcaldwel at wfubmc.edu>
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