[CIS PIDD] [cis-pidd] APECED and ARDS

[CIS-PIDD]

Hi all, we are caring for a 13yo girl with APECED who was admitted a week ago with complaints of neck and jaw pain and global myalgias w minimally elevated inflammatory markers (CRP/ESR). She was noted to have oral lesions appear 2 days later that were HSV1 (+) by PCR and her mucosa appeared macerated with lesions reminiscent of yeast, although it was culture negative. She was treated with IV acyclovir and IV diflucan but developed tachypnea and a need for oxygen with diffuse pulmonary infiltrates. Her antibiotic coverage was expanded for 48 hours and she improved. Nothing bacterial grew from blood cultures so the antibiotics were stopped and acyclovir/diflucan continued. Her CXR did not improve much but she was less tachypnic. Unfortunately she also developed a rising Cr so we were very eager to stop any nephrotoxic drugs.  Within 48-72 hours of stopping antibiotics her pulmonary status worsened again and CXR looks near "white out" reminiscent of ARDS. She is now intubated and going for bronchoscopy to determine if we are missing an organism.
I am concerned that she has ARDS secondary to an immune mediated pathology. Has anybody seen immune mediated ARDS in patients with APECED? The treatment would obviously mean using anti-inflammatory medications (probably would start with high dose IVIG and steroids). I am hesitant to do this in light of her HSV1 in her mouth and worried about giving IVIG In the setting of ARDS.
This child is relatively new to me. She was cared for largely by an endocrinologist in FL for years before coming to us in Cincinnati this winter. She was previously tested for anti:BFPIFB1 (minimally elevated) and anti:KCNRG (negative) by Dr. Lionakis (NIH) and she received rituximab in Sept 2015 (4 weeks) followed by daily Imuran treatment (NIH protocol). Her B cells vanished appropriately in the fall 2015 and are now detectable again, though low. She has all of the autoimmune endocrinopathies found in these patients. She also has autoimmune urticaria that improved after rituximab in the fall and then returned a month ago (coincident w b cells returning). In February when she was healthy, she had CT chest that did not show evidence of ILD, normal bronchoscopy and abnormal pulmonary function testing with FEV1 of 65-70% w minimal response to albuterol (moderate obstruction, no diffusion defect). The CT was improved compared to pre-rituximab (by report only as the old CT are in FL) but FEV1 is the same.

Appreciate any feedback you may have, Kim

Kimberly Risma MD PhD
Associate Professor
Allergy/Immunology
Cincinnati Children's Hospital Medical Center


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