Pediatrics International

(2003)

45

, 216–218

Patient Report

Pigeon-breeder’s disease in a child with selective IgA deficiency

EBRU YALÇIN,

1

NURAL KG

PER,

1

AYHAN GÖÇMEN,

1

U}

UR ÖZÇELG

K,

1

DENG

Z DO}

RU

1

AND ZEYNEP MISIRLIGG

L

2

1

Chest Diseases Unit, Department of Pediatrics, Hacettepe University Faculty of Medicine and

2

Department of Chest Diseases, Ankara University Faculty of Medicine

Key words

child, deficiency, IgA, lung, Pigeon-breeder’s disease.

Hypersensitivity pneumonitis, or extrinsic allergic alveolitis,is an allergic disease resulting from the sensitization andrecurrent exposure to any of a wide variety of inhaled organicdusts.

1

Pigeon-breeder’s disease (PBD) is the most commonform of this disease, but it occurs rarely in childhood.

2

Selective IgA deficiency is the most common primaryimmunodeficiency disorder. There is increased associationwith recurrent sinopulmonary infection, bronchiectasis, andauto-immune disease.

3

In the present report, we describe acase with PBD who had selective IgA deficiency. To ourknowledge this is the first case with such an association.

Case report

A 5.5-year-old girl presented with an 8-month history ofprogressive cough, dyspnea, fever, and lack of appetite. Shewas referred to our hospital from an outside hospital, withthe probable diagnosis of cystic fibrosis. Initial physicalexamination revealed diffuse crackles in the lungs bilaterallyand clubbing. The rest of the physical examination wasunremarkable. In the laboratory work-up, a chest X-rayshowed bilateral peribronchial thickening (Fig. 1). Sweatchloride test,

α

1

-antitrypsin level were normal, and arterialoxygen saturation was 95%. Pulmonary function tests werenot performed because the patient did not co-operate. Serumlevels of IgG, IgG subgroups, IgM and CH

50

, and nitroblue-tetrazolium test were normal, while serum IgA level was verylow (< 6 mg/dL). A high-resolution computerized tomography(HRCT) scan of the chest showed bilateral diffuse ground-glass appearance, disseminated centrilobular densities and airentrapments in the lungs (Fig. 2). Echocardiography revealedfindings consistent with pulmonary hypertension. Regarding

those clinical findings and radiologic abnormalities, an openlung biopsy was planned. At this time, the patient’s historywas carefully checked again, and we learned that thepatient’s father had been breeding pigeons for many years.However, the patient was not exposed to pigeons directly, butindirectly by way of her father’s clothes. For this reason, adiagnosis of PBD was considered. Precipitins were stronglypositive for pigeon antigens in the patient’s serum. Exposureto pigeon antigens was stopped, and she was treated withprednisolone (2 mg/kg, daily, p.o) for a period of 4 weeks.One month later, excellent clinical response was achieved.Three months later HRCT findings improved significantly(Fig. 3) and echocardiographic findings of pulmonary hyper-tension improved. The prednisolone dose was reduced to1 mg/kg per day.

Discussion

Pigeon-breeder’s disease in children was first described byReed

et al

.

4

It occurs after repeated inhalation of organicparticulate antigens, 4–5

µ

m in diameter, from pigeons.

6

Thedisease involves the terminal bronchioles, interstitium, andalveoli in particular.

1,3

It may appear in two distinctforms.

1,2,4,5

In the acute form, dry cough, dyspnea, and feverbegin 4–6 h after exposure to birds. The chronic form occurswhen less intense and continuous exposure exist. Cough anddyspnea may be progressive without acute episodes in thisform. Pulmonary hypertension, which is initially reversible,occurs in the early stages of the illness, becoming irreversiblewith chronic exposure due to irreversible pulmonaryfibrosis.

6

Diagnostic criteria include antigen exposure,symptoms, clinical and radiologic findings, and serologictests of precipitating antibodies.

7

Initially, we thought that the cause of chronic respiratorydisease was selective IgA deficiency. We also planned toperform an open-lung biopsy in order to explain the unex-pectedly severe thoracic CT appearances. However, when thepatient’s history was reviewed in detail, we considered PBD

Correspondence: Ebru Yalçg

n MD, Pediatrician, Chest DiseasesUnit, Department of Pediatrics, Hacettepe University Faculty ofMedicine, 06100-Ankara, Turkey. Email: ebruy@hacettepe.edu.tr

Received 5 April 2002; revised 14 August 2002; accepted26 September 2002.

Pigeon-breeder’s disease and IgA deficiency 217

as the most probable diagnosis, and the positivity of serumprecipitins for pigeon supported the diagnosis. The adminis-tration of steroids improved the patient’s symptoms as wellas the findings of pulmonary hypertension, and alsoprevented pulmonary worsening.

Investigations have established that in PBD, symptomaticpatients produced IgG and IgM antibodies against the environ-mental antigens that incite the alveolitis. This finding led to

the hypothesis that PBD is considered to be an immune-complex disorder.

8

Recent observations have indicated thatthe presence of immune complexes in the airways can induceneutrophilic infiltration through the induction of pro-inflammatory cytokines by bronchoalveolar cells.

9

However, to our knowledge, the association of IgAdefficiency and PBD has not been reported previously. Inprevious reports serum IgA levels were not investigated.

6

Itmay also be possible that in subjects with selective IgAdeficiency the failure to exclude antigens is similar to theceliac disease which is highly associated with selective IgAdeficiency.

10

Although we could not study secretory IgAlevels in the present patient, we considered that IgAdeficiency (in the absence of secretory IgA) might havefacilitated the transportation of pigeon antigens to thepatient’s respiratory units, followed by their absorption.

3,10

Inthis way, IgA deficiency might have predisposed the onset ofPBD in the present patient. However, whether IgA deficiencypredisposes to PBD requires a more comprehensive studywith an increased number of patients.

PBD should be considered in any child with recurrent orpersistent respiratory symptoms, even if the patient has IgAdeficiency. Because IgA deficiency might be a risk factor fordeveloping PBD, serum IgA levels should be determined inchildren with PBD.

References

1 Fink JN. Hypersensitivity pneumonitis.

J. Allergy Clin.Immunol.

1984;

74

: 1–10.

Fig. 1

Plain chest X-ray shows bilateral peribronchial thickening.

Fig. 2

High-resolution CT of the chest showed bilateral diffuseground-glass appearance, disseminated centrilobular densitiesand air entrapments in the lungs.

Fig. 3

High-resolution CT of the chest showed bilateral centri-lobular densities in the lungs.

218 E Yalçg

n

et al

.

2 Dilber E, Özçelik U, Göçmen A, Mg

sg

rlg

gil Z, Kiper N.Recurrent pneumonitis in a 10-year-old girl with pigeonbreeder’s disease.

Turk. J. Pediat

. 1997;

39

: 541–5.3 Ammann AJ, Stiehm ER. Antibody (B-Cell) immuno-

deficiency disorders. In: Stites DP, Terr AI, Parslow TG,(eds).

Medical Immunology

. Appleton & Lange, Connecticut1997; 332–44.

4 Reed CE, Sosman A, Barbee RA. Pigeon breeder’s lung.

JAMA

1965;

193

: 81–5.5 Stiehm ER, Reed CE, Tooley WH. Pigeon breeder’s lung in

children.

Pediatrics

1967;

39

: 904–15.6 Grech V, Vella C, Lenicker H. Pigeon breeder’s lung in

childhood.

Pediatr. Pulmonol.

2000;

30

: 154–8.

7 Aigner K, Bolitschek J, Wurtz J. Diagnostic process ofalveolitis-state of the art.

Acta Med. Austriaca

1994;

21

: 95–9.8 Amin R, Wilmott R. Hypersensitivity pneumonitis and eosino-

philic pulmonary disease. In: Chernick V, Kendig, EL, (eds).

Kendig’s Disorders of the Respiratory Tract in Children

, 6thedn. W.B. Saunders Company, Philadelphia 1998; 731–53.

9 Warren JS. Intrapulmonary interleukin 1 mediates acuteimmunocomplex alveolitis in the rat.

Biochem. Biophys. Ren.Commun.

1991;

175

: 604–10.10 Cataldo F, Marino V, Bottaro G

et al.

Celiac disease andselective immunoglobulin A deficiency.

J. Pediatr.

1997;

131

:306–8.