Abstract: | Background—In several diseases there is a relationbetween deficiency of neutrophil granulocytes and granulomatouslesions. Recently, in glycogen storage disease type Ib, this relationhas been supported by the beneficial effect of treatment of enteritis with granulocyte-macrophage colony stimulating factor. Aim—To investigate whether chronic granulomatousdisease could be treated according to the same principle. Patients and methods—Inflammatory lesions weremonitored in two brothers with chronic granulomatous diseasedemonstrated by very low superoxide production in neutrophilgranulocytes. The two patients were treated with recombinant humangranulocyte colony stimulating factor on three occasions when thedisease was active. Results—In one patient, remission of an inflamedstenosis of the colon sigmoideum was shown by granulocytescintigraphy after one month of treatment with granulocyte colonystimulating factor. In the other patient, remission of colon diseaseand later of a non-malignant tumour in the right lung hilum was shownby colonoscopy and computed tomography scans respectively. Conclusion—Remission of inflammatory lesions intwo brothers with chronic granulomatous disease was induced bygranulocyte colony stimulating factor on three occasions. The mechanismfor 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.
Keywords:chronic granulomatous disease; enteritis; granulocyte colony stimulating factor |