Abstract

Background—In several diseases there is a relation between deficiency of neutrophil granulocytes and granulomatous lesions. Recently, in glycogen storage disease type Ib, this relation has been supported by the beneficial effect of treatment of enteritis with granulocyte-macrophage colony stimulating factor.

Aim—To investigate whether chronic granulomatous disease could be treated according to the same principle.

Patients and methods—Inflammatory lesions were monitored in two brothers with chronic granulomatous disease demonstrated by very low superoxide production in neutrophil granulocytes. The two patients were treated with recombinant human granulocyte colony stimulating factor on three occasions when the disease was active.

Results—In one patient, remission of an inflamed stenosis of the colon sigmoideum was shown by granulocyte scintigraphy after one month of treatment with granulocyte colony stimulating factor. In the other patient, remission of colon disease and later of a non-malignant tumour in the right lung hilum was shown by colonoscopy and computed tomography scans respectively.

Conclusion—Remission of inflammatory lesions in two brothers with chronic granulomatous disease was induced by granulocyte colony stimulating factor on three occasions. The mechanism for this effect is not known. The result is similar to the response found in patients with leucocyte deficiency due to glycogen storage disease type Ib.