Prophylactic therapy for selective IgA deficiency

Prophylactic therapy for selective IgA deficiency

THE LANCET spontaneous cough at night has been connected with the electroencephalographic sleep stage in patients with chronic bronchitis.3 However, ...

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spontaneous cough at night has been connected with the electroencephalographic sleep stage in patients with chronic bronchitis.3 However, our findings suggest that cough reflex may protect against retention of bronchial secretions during the night in patients with productive cough even though sleep itself may be disturbed by nocturnal cough in such patients. 1

2 3

Kuhn JJ, Hendley JO, Adams KF, Clark JW, Gwaltney JM. Antitussive effect of guaifenesin in young adults with natural colds: objective and subjective assessment. Chest 1982; 82: 713–18. Shann F. How often do children cough? Lancet 1996; 348: 699–700. Power JT, Stewart IC, Connaughton JJ, et al. Nocturnal cough in patients with chronic bronchitis and emphysema. Am Rev Respir Dis 1984; 130: 999–1001.

Department of Geriatric Medicine, Tohoku University School of Medicine, Sendai 980, Japan (H Sasaki)

Prophylactic therapy for selective IgA deficiency

gradually decreased and could not be detected after 40 months. In the second patient, the IgA antibody value remained low. None of the remaining eight patients developed detectable IgA antibodies. Thus far, a total of 1707 infusions have been given and only six (0·4%) mild systemic adverse reactions (headache, nausea, and/or light dizziness) have been noted. Our preliminary experience indicates that prophylactic IgG replacement therapy can safely be given to patients with selective IgA deficiency including those with IgA antibodies by subcutaneous administration. The treatment seems to be effective as the frequency of bacterial respiratory tract infections was reduced. One possible explanation why administration of IgG is effective in IgA deficiency could be that the patients are given additional specific antibodies that they themselves are unable to produce in sufficient amounts. According to the estimated prevalence, the number of IgA deficient individuals is 20 000 in Sweden, 120 000 in UK, and at least 250 000 in the USA. Given that 30% of these have an increased susceptibility to infections, further studies are needed to decide whether to broaden the indications for administration of subcutaneous IgG.

R Gustafson, A Gardulf, C Granert, S Hansen, L Hammarström 1

Selective IgA deficiency is the most common primary immunodeficiency with an estimated prevalence of 1/500 in whites. About 30% of these individuals have frequent bacterial respiratory tract infections affecting their daily living.1 The deficiency may be selective or combined with an IgG-subclass deficiency 2 or an impaired production of antibodies against bacterial polysaccharide antigens. 3 Prophylactic IgG replacement therapy has been contraindicated in these patients because of the risk of severe systemic adverse reactions and/or the development of IgA antibodies. Replacement IgG with rapid subcutaneous infusions has proved to be a safe and effective method to treat patients with hypogammaglobulinaemia or IgG-subclass deficiencies with or without concomitant IgA deficiency4,5 and also patients with high titres of IgA antibodies.5 We studied whether IgG therapy was useful in prophylaxis of infections in patients with selective IgA deficiency. Ten patients (five men; mean age 43 [26–61] years) were enrolled for a pilot study. Inclusion criteria were: serum IgA below 0·05 g/L, IgG and IgM within normal range, no coexisting IgG subclass deficiency, or detectable impaired production of antibodies against polysaccharide or protein antigens, lack of secretory IgA and at least four bacterial infections per year requiring antibiotic treatment for at least 3 years before initiation of therapy. The patients selfadministered weekly subcutaneous IgG infusions at home (dose of 100 mg/kg per week; Gammanorm, Pharmacia & Upjohn, n=7; or Immunoglobulin, Immuno AG, n=3). 4,5 At the time of evaluation, the patients had been treated for 18–43 months. Data about infection frequency were compiled for each patient for the past 12 months of treatment. All patients had a reduced frequency of infections when treated: No of infections per year

No of patients before IgG

No of patients after IgG

0–1 2–3 肁4

0 0 10

5 5 0

Two patients had IgA antibodies before therapy was started (high and low concentrations, respectively). In the first patient, the high concentration of IgA antibodies

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Buckley RH. Clinical and immunologic features of selective IgA deficiency. Birth Defects 1975; 11: 134–42. Oxelius V-A, Laurell A-B, Lindquist B, et al. IgG subclasses in selective IgA deficiency—importance of IgG2-IgGA deficiency. N Engl J Med 1981; 304: 1476. Hammarström L, Persson MAA, Smith CIE. Immunoglobulin subclass distribution of human anti-carbohydrate antibodies: aberrant pattern in IgA-deficient donors. Immunology 1985; 54: 821–26. Gardulf A, Hammarström L, Smith CIE. Home treatment of hypogammaglobulinaemia with subcutaneous gammaglobulin by rapid infusion. Lancet 1991; 338: 162–66. Gardulf A, Andersen V, Björkander J, et al. Subcutaneous immunoglobulin replacement in patients with primary antibody deficiencies: safety and costs. Lancet 1995; 345: 365–69.

Department of Immunology, Microbiology, Pathology and Infectious Diseases, Immunodeficiency Unit at the Division of Clinical Immunology (R Gustafson), Division of Infectious Diseases, Karolinska Institute; and Nursing Research and Development Unit, Huddinge University Hospital, Stockholm, S-14186 Huddinge, Sweden

Creutzfeldt-Jakob disease with florid plaques after cadaveric dural graft in a Japanese woman Shutaro Takashima, Jun Tateishi, Yoshiharu Taguchi, Hiroshi Inoue

The Japanese National Committee for Creutzfeldt-Jakob disease (CJD) reported there are 43 patients with CJD in Japan after implantation of cadaveric dura mater, and that no case of new-variant CJD (nvCJD) 1 has been detected. We report a case with similar features to nvCJD. At the age of 38 years, a Japanese woman received a cadaveric dural graft (Lyodura, B Braun Melsungen AG, Germany) after a clipping procedure of the right middle cerebral artery aneurysm in September, 1985. In November, 1994, 110 months after the procedure, she became unsteady and had blurred vision. She was admitted to hospital because of progressing ataxia. Examination disclosed dementia, visual disturbance, hyperreflexia, and cerebellar ataxia. Blood chemistry and cerebrospinal fluid were normal. Brain computed tomography (CT) showed no abnormal lesions. There was slow activities without periodic discharge on her electroencephalogram (EEG).