Introduction: Celiac disease (CD) is a small bowel autoimmune enteropathy mediated by antibodies against dietary gluten. We present a case of a patient with multiple sclerosis with persistently positive IgA antibodies against deamidated gliadin peptide (DGP) who was ultimately demonstrated not to have CD after decades of gluten avoidance. Case Description/Methods: A 64-year-old male with a history of multiple sclerosis (MS) and a family history of celiac disease in his mother presented to clinic to clarify his CD diagnosis. On prior testing he had positive DGP IgA (39 U) and weakly positive tissue transglutaminase (tTG) IgG serologies (8 U/mL, upper limit of normal 5 U/mL). However, prior esophagogastroduodenoscopy (EGD) was reportedly unremarkable. Based on serologies, he had been told he had CD and followed a gluten-free diet for years. On presentation, he denied bloating, diarrhea, abdominal pain, or weight changes. Labs were notable for an elevated DGP IgA level of 38 U with normal DGP IgG, tTG IgA, and tTG IgG serologies. On EGD while on a gluten free diet, the duodenum appeared normal, and pathology showed normal duodenal mucosa without villous atrophy (VA). Studies were repeated after several months of dietary gluten reintroduction. DGP IgA remained persistently elevated at 37 U, DGP IgG and tTG IgA remained within normal limits, and tTG IgG was borderline at 6 U/mL, of questionable significance. Genotyping was positive for HLA-DQ8 but negative for HLA-DQ2. Repeat EGD after gluten reintroduction demonstrated a normal-appearing duodenum without evidence of VA on pathology. The patient was informed that he did not have celiac disease. Discussion: Serologies in CD can be impacted by autoimmune disease. This patient had MS-related immunogenicity to DGP in the absence of CD. Several studies have noted that patients with MS without CD often have higher titers of DGP IgA antibodies, which may be due to antibody cross reactivity or even increased gut permeability to DGP. When serology is discordant, as in this case, duodenal biopsy should be performed, ideally after a gluten challenge. If repeat biopsy and tTG IgA serologies remain negative after gluten challenge, CD is highly unlikely. However, these patients should be closely followed for development of symptoms of CD, as latent CD is also possible.
Introduction: Cryoglobulinemic vasculitis (CV) is caused by cryoglobulins that bind to polyclonal IgG and can damage multiple organ systems. It is associated with active hepatitis C virus (HCV) infections and the primary therapy is usually treatment of the underlying HCV. We present a rare case of CV occurring in a post-liver transplant (LT) patient who was treated for HCV with sustained virologic response (SVR) for more than 6 years. Case Description/Methods: A 67-year-old with history of HCV cirrhosis and hepatocellular carcinoma treated with LT in 2011 on tacrolimus, chronic kidney disease stage 3, hypertension, hyperlipidemia initially presented to an outside hospital with nausea, vomiting, and diarrhea which resolved with supportive care. She later developed arthralgias, a maculopapular rash, and left eye pain with orbital swelling. She was evaluated by ophthalmology without findings of retinitis but with bilateral elevated intraocular pressures. MRI orbits revealed lacrimal gland adenititis treated with topical medications including erythromycin and systemic acetazolamide. Dermatology evaluated patient with a skin punch biopsy which showed vascular/perivascular C3 and fibrinogen staining suggestive of early vasculitis without evidence of IgA vasculitis. Notably, she was treated with ledipasvir/sofosbuvir and ribavirin which was completed 7/2015 with SVR achieved 12/2015. HCV RNA viral load was again negative as well as other viruses. Due to rapidly worsening renal function with creatinine rising to 3.8 from baseline of 1.3-1.5, a renal biopsy was performed which revealed cryoglobulinemic glomerulonephritis-establishing a diagnosis of CV. Patient's course was complicated by a biopsy-related renal hematoma requiring embolization. She was treated with pulse dose solumedrol and plasmapheresis. Shortly after treatment, her rash, vision, and arthralgias improved. Her creatinine returned to baseline and she was started on prednisone 60mg daily. Discussion: CV is typically associated with active HCV infection, but in rare cases can be found in treated patients with SVR. This case is also unique in that it involves a post-LT patient on tacrolimus, raising the question of the impact of tacrolimus in developing CV in a patient with SVR. Moreover, multiple modalities of diagnosis may need to be pursued if initial biopsies are not conclusive to establish effective treatment plans. Overall, CV should remain on the differential in patients with a treated HCV and SVR.
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