Crohnʼs Disease or Wegenerʼs Granulamatosis or Both?

Abstract

Purpose: A 36-year-old Hispanic male with a recent diagnosis of Crohn's disease came to our tertiary care center for further management of his care. This patient had frequent flares of Crohn's despite being on maintainence mesalamine therapy and adequate treatment of his flares with a standard prednisone dose. He had failed 6 MP treatment in the past due to liver toxicity and Remicade treatment due to severe anaphylaxis. During the course of his disease he had recurrent episodes of C. diff colitis which were appropriately treated. Patient in addition to features of colitis presented with other extraintestinal findings at various time points. These findings included neutrophilic vasculitis and skin abscesses proved on skin biopsy, sinusitis and nasal polyps proved on CT sinus and ENT evaluation, oral ulcers,pulmonary granulomatous nodules on CT chest, granulomas on his bone marrow biopsy, granulomas on his spleen on CT abdomen, uveitis on ophthalmology evaluation,proteinuria and hematuria on urinalysis and non-specific arthralgias and arthritis. After a very thoughtful analysis of these symptoms, we decided to perform a detailed rheumatological work-up. Our top differential diagnoses included WG and Behcet's disease. The patient was found to be ANCA negative but strongly positive for proteinase 3 antibody. Proteinase 3 antibody is a very specific antibody for WG. There are several diagnostic criterias that have been proposed for WG including American College of Rheumatology (ACR) criteria, criteria by Chapel Hill Consensus Conference and European Medicines Agency algorithm but serological criteria over rides all clinical criterias. Based on the work-up described above, we decided to treat the patient's acute colitis the same way as we would treat WG. Patient was treated with high dose solumedrol, which is typically not used to treat Crohn's disease flares, as well with rituximab. Patient had very good clinical response to this treatment regimen thereby further confirming the suspicion of WG associated colitis. Conclusion: There are less than five case reports reported in the medical literature where WG was found to co-exist with Crohn's disease. Those reviewed included cutaneous WG in patient with Crohn's disease, granulomatous gastritis in WG and colonic manifestations mimicking Crohn's disease in WG. Amongst all these studies serological work-up was key for diagnosis of WG. WG should be considered a diagnostic possibility in patients who present with new symptoms that are not typical of Crohn's disease. This may help immensely to accurately diagnose the underlying true disease and planning optimal management strategies.