P-001 Crohn’s-like Disease in a Patient with Good Syndrome

Abstract

There has been described a Crohn’s-like disease in patients with congenital immunodeficiency syndromes (chronic granulomatous disease, glycogen storage disease type Ib, common variable immunodeficiency…). We report a case of a Crohn’s-like colitis and perianal fistula in a patient with Good Syndrome, an acquired immunodeficiency. A 58 years old male, was diagnosed in January 2004 of thymoma and secondary hypogammaglobulinemia (Good Syndrome). He was treated by thymectomy and radiotherapy. Eleven months later the surgery, the patient developed bloody diarrhea, colicky abdominal pain, fever, weight loss and complex perianal fistula. In physical exam, abdomen exploration was not remarkable; there was a perianal fistula with fecal and purulent drainage. The laboratory findings were: hemoglobin 12.4 g/dL, platelets 70 × 10^9, CD4 293 cells/μL, CD3 and CD8 were normal, ratio CD4/CD8 0.34, IgA 14 mg/dL and IgM 5 mg/dL. The rest of results were normal. A colonoscopy was carried out finding multiple superficial ulcers in rectum, sigma, left colon and cecum. Transverse, right colon and ileum were unaffected. Biopsies showed nonspecific moderate inflammatory infiltrate of lymphocytes and plasma cells. No granulomas, crypt abscesses or CMV inclusions were found. Fecal cultures were all negative. Finally a probability diagnosis of Crohn’s disease was done and the patient was treated with mesalazine, steroids and metronidazole without any improvement. Monthly IV immunoglobulins were also administered. Thiopurines and anti-TNF were not used because of recent diagnosis of thymoma. During the following 5 years progressive pancytopenia was developed and recurrent fungal, bacterial and viral infections occurred including a septic shock. Finally the patient died in February 2009 because of E. coli septicemia. Thymomas represent 0.2%–1.5% of malignant neoplasias; 3%–6% of them develop hypogammaglobulinemia after the surgery, what it is called Good syndrome. This acquired immunodeficiency is both cellular and humoral; Immunoglobulins are absent or very low and CD4 are low with a CD4/CD8 ratio <1. The patient suffers different recurrent and opportunistic infections; being the general survival 4 years. There is no specific treatment despite IV immunoglobulins therapy. A Crohn’s-like disease has been reported in other immunodeficiency syndromes (as a chronic granulomatous disease, glycogen storage disease type Ib or common variable immunodeficiency). Our patient had a phenotypically Crohn’s disease pattern, but biopsy was not specific and he did not have a response after steroids treatment. We hypothesize that this patient had a Crohn’s-like syndrome similar to those have been reported in other immunodeficiencies. Congenital immunodeficiency syndromes sometimes developed a Crohn’s-like syndrome. - Good syndrome is an acquired immunodeficiency syndrome compromising both cellular and humoral immune response. - We describe a Crohn’s-like syndrome on a patient with Good syndrome, suggesting a similar pathogenic mechanism with congenital immunodeficiency and possibly Crohn’s disease.