Correspondence

A Fractured Diagnosis

N Engl J Med 2005; 353:1747October 20, 2005

Article

To the Editor:

Cukierman et al., in their Clinical Problem-Solving article (Aug. 4 issue),1 affirm that “the clinical picture suggests primary Sjögren's syndrome because of the lack of clinical features associated with rheumatoid arthritis or other autoimmune disorders,” according to the revised European criteria.2 The more recent American–European Consensus Group classification3 establishes as exclusion criteria for primary Sjögren's syndrome the presence of infection with hepatitis C virus (HCV) or the human immunodeficiency virus (HIV). Were HCV and HIV infection considered in this patient? Is there another fragment in the fractured diagnosis?

Francisco López-Medrano, M.D.
Manuel Lizasoain, M.D.
José María Aguado, M.D., Ph.D.
University Hospital 12 de Octubre, 28041 Madrid, Spain
flmedrano@yahoo.es

3 References

1. 1

   Cukierman T, Gatt ME, Hiller N, Chajek-Shaul T. A fractured diagnosis. N Engl J Med 2005;353:509-514
   Full Text | Web of Science | Medline

2. 2

   Vitali C, Bombardieri S, Moutsopoulos HM, et al. Preliminary criteria for the classification of Sjogren's syndrome: results of a prospective concerted action supported by the European Community. Arthritis Rheum 1993;36:340-347
   CrossRef | Web of Science | Medline

3. 3

   Vitali C, Bombardieri S, Jonsson R, et al. Classification criteria for Sjogren's syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis 2002;61:554-558
   CrossRef | Web of Science | Medline

Author/Editor Response

The revised American–European Consensus Group classification scheme requires that before the diagnosis of primary Sjögren's syndrome be made, the following conditions must be ruled out: infection with HCV or HIV, lymphoma, sarcoidosis, graft-versus-host disease, and the use of anticholinergic drugs.

Our patient was tested for HCV and HIV and was negative for both. Therefore, the diagnosis of primary Sjögren's syndrome is still plausible. Despite this fact, it is possible that longer follow-up might reveal another underlying diagnosis.

An increasing body of evidence supports a strong association between HCV infection and a Sjögren's syndrome–like condition. In one study, 12 percent of patients with Sjögren's syndrome were HCV-positive.1 In another study, 25.9 percent of people with chronic HCV infection met the European criteria for Sjögren's syndrome.2 Clinically, primary Sjögren's syndrome may be differentiated from Sjögren's syndrome associated with HCV infection by the absence of neuropathy, vasculitis, or liver involvement, as well as by the much higher frequency of antibodies to Ro and La.1

Tali Cukierman, M.D.
Nurith Hiller, M.D.
Tova Chajek-Shaul, M.D.
Hadassah–Hebrew University Medical Center, Mount Scopus, Jerusalem 91240, Israel
chajek@hadassah.org.il

2 References

1. 1

   Ramos-Casals M, De Vita S, Tzioufas AG. Hepatitis C virus, Sjogren's syndrome and B-cell lymphoma: linking infection, autoimmunity and cancer. Autoimmun Rev 2005;4:8-15
   CrossRef | Web of Science | Medline

2. 2

   Nagao Y, Hanada S, Shishido S, et al. Incidence of Sjogren's syndrome in Japanese patients with hepatitis C virus infection. J Gastroenterol Hepatol 2003;18:258-266
   CrossRef | Web of Science | Medline