A MISLEADING DIAGNOSIS OF HYPERSENSITIVITY PNEUMONITIS IN A PATIENT SUFFERING FROM AN ASPERGILLUS INFECTION AND AUTOSOMAL CHRONIC GRANULOMATOUS-DISEASE (CGD) - JUST ONE EXAMPLE OF A DIAGNOSTIC TRAP TYPICAL FORTHIS KIND OF CGD
E. Raeder et al., A MISLEADING DIAGNOSIS OF HYPERSENSITIVITY PNEUMONITIS IN A PATIENT SUFFERING FROM AN ASPERGILLUS INFECTION AND AUTOSOMAL CHRONIC GRANULOMATOUS-DISEASE (CGD) - JUST ONE EXAMPLE OF A DIAGNOSTIC TRAP TYPICAL FORTHIS KIND OF CGD, Monatsschrift fur Kinderheilkunde, 145(8), 1997, pp. 810-814
Chronic granulomatous disease (CGD) is caused by the inability of phag
ocytes to kill ingested germs by microbicidal reactive oxygen. Especia
lly forms of CGD with retained ability to produce some reactive oxygen
sometimes tend to imitate other diseases. An 8-year-old girl with a n
ormal history presented with many symptoms matching those of hypersens
itivity pneumonitis like restrictive ventilation disorder, suggestive
chest x-ray, a majority of CD8 positive lymphocytes in the broncho-alv
eolar-lavage fluid (BAL), suggestive histological valuation. Hints for
the true underlying disease like neutrophilia and a high anti-Aspergi
llus-titer could be overlooked easily. The patient was treated with st
eroids and antibiotics because an infection could not be excluded. The
CGD had not been recognised at this time, however. After a significan
t improvement for a short time the patient experienced a dramatic resp
iratory deterioration. A lot of Aspergillus could now be found in the
BAL. Finally the patient died because of an ARDS and respiratory insuf
ficiency even though signs of the Aspergillus infection improved signi
ficantly. Discussion: Whenever CGD must be considered appropriate diag
nostics have to be performed-especially before treatment with steroids
.