Possible primary immunodeficiency presenting with gastrointestinal symptoms: Case report and minireview

Wang,Honggang; Wang,Min; Fan,Zhining; Ji,Guozhong; Zhang,Faming

doi:10.3892/etm.2013.1178

Possible primary immunodeficiency presenting with gastrointestinal symptoms: Case report and minireview

Authors:
- Honggang Wang
- Min Wang
- Zhining Fan
- Guozhong Ji
- Faming Zhang
View Affiliations

Affiliations: Institute of Digestive Endoscopy and Medical Center for Digestive Diseases, Second Affiliated Hospital of Nanjing Medical University, Nanjing, Jiangsu 210011, P.R. China
Published online on: June 25, 2013 https://doi.org/10.3892/etm.2013.1178
Pages: 616-618

Metrics: Total Views: 0 (Spandidos Publications: | PMC Statistics: )

Metrics: Total PDF Downloads: 0 (Spandidos Publications: | PMC Statistics: )

Cited By (CrossRef): 0 citations Loading Articles...

Abstract

Primary immunodeficiency is a disease characterized by reduced levels of serum immunoglobulins and multiple clinical manifestations. Patients with primary immunodeficiency frequently present with gastrointestinal symptoms, such as diarrhea, malabsorption and weight loss. The mainstay of treatment is replacement therapy with intravenous immunoglobulin (IVIG). In the current study, we report the case of a 23‑year‑old man with symptoms of chronic diarrhea, malabsorption and weight loss that had been apparent for two years. Subsequent to being diagnosed with possible primary immunodeficiency, the patient was treated with 30 mg/day oral prednisone for one month. The prednisone was then tapered weekly by 5 mg until withdrawal. Three months later, the patient's clinical symptoms disappeared and his quality of life improved. During the subsequent nine months follow‑up, the patient was able to work without suffering any effects from his illness. The body weight of the patient increased and plasma albumin levels were normal. In conclusion, this study describes the case of a patient with primary immunodeficiency‑related gastrointestinal symptoms who responded well to oral prednisone treatment.

View Figures

View References

1.	Lim MS and Elenitoba-Johnson KS: The molecular pathology of primary immunodeficiencies. J Mol Diagn. 6:59–83. 2004. View Article : Google Scholar : PubMed/NCBI
2.	Conley ME, Notarangelo LD and Etzioni A; Representing PAGID (Pan-American Group for Immunodeficiency) and ESID (European Society for Immunodeficiencies): Diagnostic criteria for primary immunodeficiencies. Clin Immunol. 93:190–197. 1999. View Article : Google Scholar : PubMed/NCBI
3.	Porta F, Forino C, De Martiis D, et al: Stem cell transplantation for primary immunodeficiencies. Bone Marrow Transplant. 41(Suppl 2): S83–S86. 2008. View Article : Google Scholar
4.	Boyle JM and Buckley RH: Population prevalence of diagnosed primary immunodeficiency diseases in the United States. J Clin Immunol. 27:497–502. 2007. View Article : Google Scholar : PubMed/NCBI
5.	Wood P, Stanworth S, Burton J, et al: Recognition, clinical diagnosis and management of patients with primary antibody deficiencies: a systematic review. Clin Exp Immunol. 149:410–423. 2007. View Article : Google Scholar : PubMed/NCBI
6.	Lai Ping So A and Mayer L: Gastrointestinal manifestations of primary immunodeficiency disorders. Semin Gastrointest Dis. 8:22–32. 1997.PubMed/NCBI
7.	Kobrynski LJ and Mayer L: Diagnosis and treatment of primary immunodeficiency disease in patients with gastrointestinal symptoms. Clin Immunol. 139:238–248. 2011. View Article : Google Scholar : PubMed/NCBI
8.	Elnachef N, McMorris M and Chey WD: Successful treatment of common variable immunodeficiency disorder-associated diarrhea with budesonide: a case report. Am J Gastroenterol. 102:1322–1325. 2007. View Article : Google Scholar
9.	Park MA, Li JT, Hagan JB, et al: Common variable immunodeficiency: a new look at an old disease. Lancet. 372:489–502. 2008. View Article : Google Scholar : PubMed/NCBI
10.	Washington K, Stenzel TT, Buckley RH and Gottfried MR: Gastrointestinal pathology in patients with common variable immunodeficiency and X-linked agammaglobulinemia. Am J Surg Pathol. 20:1240–1252. 1996. View Article : Google Scholar : PubMed/NCBI
11.	Sneller MC: Common variable immunodeficiency. Am J Med Sci. 321:42–48. 2001. View Article : Google Scholar
12.	Ammann AJ, Ashman RF, Buckley RH, et al: Use of intravenous gamma-globulin in antibody immunodeficiency: results of a multicenter controlled trial. Clin Immunol Immunopathol. 22:60–67. 1982. View Article : Google Scholar : PubMed/NCBI
13.	Pirofsky B: Intravenous immune globulin therapy in hypogammaglobulinemia. A review. Am J Med. 76:53–60. 1984. View Article : Google Scholar : PubMed/NCBI
14.	Cunningham-Rundles C, Siegal FP, Smithwick EM, et al: Efficacy of intravenous immunoglobulin in primary humoral immunodeficiency disease. Ann Intern Med. 101:435–439. 1984. View Article : Google Scholar : PubMed/NCBI
15.	Orange JS, Hossny EM, Weiler CR, et al: Use of intravenous immunoglobulin in human disease: a review of evidence by members of the Primary Immunodeficiency Committee of the American Academy of Allergy, Asthma and Immunology. J Allergy Clin Immunol. 117(Suppl): S525–S553. 2006. View Article : Google Scholar
16.	Lucas M, Lee M, Lortan J, et al: Infection outcomes in patients with common variable immunodeficiency disorders: relationship to immunoglobulin therapy over 22 years. J Allergy Clin Immunol. 125:1354–1360. 2010.PubMed/NCBI
17.	Hermaszewski RA and Webster AD: Primary hypogammaglobulinaemia: a survey of clinical manifestations and complications. Q J Med. 86:31–42. 1993.PubMed/NCBI
18.	Kalha I and Sellin JH: Common variable immunodeficiency and the gastrointestinal tract. Curr Gastroenterol Rep. 6:377–383. 2004. View Article : Google Scholar : PubMed/NCBI
19.	Zhang F, Luo W, Shi Y, et al: Should we standardize the 1,700-year-old fecal microbiota transplantation? Am J Gastroenterol. 107:17552012.PubMed/NCBI
20.	Borody TJ and Khoruts A: Fecal microbiota transplantation and emerging applications. Nat Rev Gastroenterol Hepatol. 9:88–96. 2012. View Article : Google Scholar : PubMed/NCBI