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Serum IgD as a discriminator between the two periodic febrile syndromes hyper- immunoglobulinaemia D syndrome and Behçet’s disease
  1. NAPHTALI BREZNIAK,
  2. SHMUEL SHTRASBURG,
  3. PNINA LANGEVITZ,
  4. AVI LIVNEH
  1. Heller Institute of Medical Research, Sheba Medical Center, Tel-Hashomer, Israel
  2. Department of Medicine, University Hospital St Radboud, Nijmegen, the Netherlands
  3. Sackler Faculty, Tel-Aviv University, Israel
  1. Dr A Livneh, Heller Institute of Medical Research, Sheba Medical Center, Tel-Hashomer 52621, Israel.
  1. JOOST P H DRENTH
  1. Heller Institute of Medical Research, Sheba Medical Center, Tel-Hashomer, Israel
  2. Department of Medicine, University Hospital St Radboud, Nijmegen, the Netherlands
  3. Sackler Faculty, Tel-Aviv University, Israel
  1. Dr A Livneh, Heller Institute of Medical Research, Sheba Medical Center, Tel-Hashomer 52621, Israel.
  1. SHMUEL SHTRASBURG,
  2. PNINA LANGEVITZ,
  3. AVI LIVNEH
  1. Heller Institute of Medical Research, Sheba Medical Center, Tel-Hashomer, Israel
  2. Department of Medicine, University Hospital St Radboud, Nijmegen, the Netherlands
  3. Sackler Faculty, Tel-Aviv University, Israel
  1. Dr A Livneh, Heller Institute of Medical Research, Sheba Medical Center, Tel-Hashomer 52621, Israel.

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The hyperimmunoglobulinaemia D (IgD) syndrome (HIDS) is a recently described periodic disease, manifested by febrile episodes, lasting several days and accompanied by abdominal pain, symmetrical oligoarthritis, cervical lymphadenopathy, skin lesions, and constantly increased serum IgD concentrations (>100 units/ml).1 HIDS shares its episodic febrile nature and many painful manifestations with several other clinical conditions, including familial Mediterranean fever, familial Hibernian fever, and Behçet’s disease (BD). Indeed, in a leading textbook of rheumatology, these entities were recently grouped in one chapter, entitled Intermittent and Periodic Arthritic Syndromes.2 Manifestations, distinguishing between these periodic syndromes, are therefore of considerable interest and thoroughly investigated.

Normal or low concentrations of serum IgD exclude HIDS. However, as about 10–15% of familial Mediterranean fever and familial Hibernian fever patients have increased concentrations of IgD (unpublished data),1 3 increased IgD concentrations cannot safely differentiate between HIDS and familial Mediterranean fever or familial Hibernian fever in a patient presenting with periodic arthritic syndrome. As for BD, comparative IgD studies with HIDS are lacking and the status of IgD as a discriminator between the diseases is not known.

We have studied IgD concentrations in 30 serum samples of patients with BD, in whom the diagnosis agreed with the International Study Group Criteria for the diagnosis of BD.4 Serum IgD concentrations were determined using a sensitive enzyme linked immunosorbent assay (ELISA).5 In this assay, rabbit anti-human IgD anti-serum was used to coat a multititre plate, followed by the sequential addition of the examined serum samples or standards, monoclonal mouse anti-human IgD, rabbit anti-mouse IgG peroxidase conjugated, and substrate. Serum samples of five healthy people and five HIDS patients served as controls.

The mean (SD) IgD serum concentrations in patients with BD and healthy controls were comparable (62.1 (36.6) compared with 60.2 (35.2) units/ml, respectively). Only one patient with BD had raised serum IgD concentrations (240 units/ml). In contrast the IgD concentrations in all HIDS serum samples were high (188.7 (144.7) units/ml, p< 0.001 by Student’s t test, compared with either healthy controls or BD patients). Our study is consistent with a previous report, showing normal serum IgD concentrations in BD.6 That report however, used a different, less sensitive methodology (immunodiffusion assay), compared BD with normal controls but not with HIDS patients, and is the only previous study we are aware of.

Our results therefore, suggest that IgD determination may facilitate the distinction between the two occasionally resembling entities, HIDS and BD. However, as high serum IgD concentrations may infrequently be found in patients with BD, the results of this test may support but not replace clinical evaluation, which still remains the mainstay of diagnosis of the various intermittent arthritic syndromes.

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