author={JosW. M. van der Meer and Jiri Radl and C. J. L. M. Meyer and Jaak Vossen and Janny A. van Nieuwkoop and Sacha Lobatto and Ralph van Furth},
  journal={The Lancet},

Hyperimmunoglobulinemia D syndrome in an Arab child

The case presented is the first from Arab countries to be registered in the International HIDS Registry and to the authors' knowledge the first to be reported.

Atypical hypergammaglobulinaemia D syndrome with amyloidosis: An overlap with familial Mediterranean fever?

A case of hypergammaglobulinaemia D and periodic fever syndrome, developing an amyloidosis-related nephrotic syndrome, is reported and an overlap syndrome between these two rare clinical disorders can be suggested.

Hyperimmunoglobulinaemia D syndrome: a rare cause of prolonged fever and treatment with anti-interleukin 1 agent

A case of 16-month-old boy who had episodes of recurrent fever accompanied by maculopapular rash and lymphadenopathy is reported, diagnosed as HIDS and he had heterozygote mutation of mevalonate kinase gene.

Hyper-IgD and periodic fever syndrome (HIDS) due to compound heterozygosity for G336S and V377I in a 44-year-old patient with a 27-year history of fever

A novel pathogenic mutation in the MVK gene is reported as the cause of fever in a 44-year-old male patient with a history of fever over a period of 27 years.

Hereditary periodic fever with systemic amyloidosis: is hyper-IgD syndrome really a benign disease?

A case of amyloidosis in association with hyperimmunoglobulinemia D syndrome with unusual complications of HIDS seriously challenge the assumption that the disease is associated with a good prognosis.

Hyper-IGD syndrome: A new case treated with colchicine

SummaryWe report a new case of hyper-IgD syndrome, a recently described disease characterized by recurrent episodes of fever with headache, bilateral cervical lymphadenopathy and, more rarely,

Hereditary periodic fever.

Three syndromes of hereditary periodic fever are discussed: familial Mediterranean fever, the hyper-IgD syndrome, and the tumor necrosis factor receptor–associated periodic syndrome.

Hyperimmunoglobulin D Syndrome in Childhood

Recent reports show promising results with anakinra and etanercept to treat the attacks of hyperimmunoglobulinemia D and periodic fever syndrome, an autoinflammatory syndrome.

Hyperimmunoglobulinemia D syndrome successfully treated with a corticosteroid

A Japanese boy with HIDS is successfully treated with corticosteroid therapy and the pathogenesis of this syndrome remains unclear and an effective therapy has not yet been established.



Cutaneous manifestations of familial Mediterranean fever.

Familial Mediterranean fever (FMF) is frequently accompanied by erysipelas-like attacks. These should alert the physician to the correct diagnosis of this systemic disease. Several other nonspecific

Familial Mediterranean fever: report of a large family, review of the literature, and discussion of the frequency of amyloidosis.

Most of the attacks, however, are associated with a rise in temperature although the fever spike may be more transient than the associated symptoms, and any individual with suggestive symptoms who is significantly disabled by the attacks should have a therapeutic trial of colchicine.

Colchicine therapy for familial mediterranean fever. A double-blind trial.

Eleven patients with long standing familial Mediterranean fever were studied in a double-blind trial using daily colchicine or placebo to determine the best course of action for each patient.


  • S. Siegal
  • Medicine
    The American journal of medicine
  • 1964

Immunoglobulin D in serum, body fluids and lymphoid tissues.

IgD levels were not increased in three examples of `autoimmune' disease, but increases were noted in some patients having chronic infections, and IgD was usually absent in hypogammaglobulinaemia.

Elevated IgD antibodies to wheat in celiac disease.

The levels of IgE or IgD antibodies to either wheat or milk showed no relationship to the presence of precipitins to the antigens of these two foods and the study did not demonstrate a role for IgE in celiac disease.

Metabolism of human immunoglobulin D (IgD).

The metabolism of labeled IgD was investigated in 28 patients selected so as to cover a wide range of immunoglobulin disorders and indicates that the synthetic rate is the major factor controlling the serum IgD concen- tion.