Non-X-linked hyperimmunoglobulin M syndrome with chronic interstitial pneumonitis
Identifieur interne : 002586 ( Main/Exploration ); précédent : 002585; suivant : 002587Non-X-linked hyperimmunoglobulin M syndrome with chronic interstitial pneumonitis
Auteurs : A. Quezada [Chili] ; X. Norambuena [Chili] ; R. Gonzalez [Chili] ; S.-M. Ramos [Chili] ; A. Chavez [Chili] ; S. Gonzalez [Chili]Source :
- Journal of investigational allergology & clinical immunology [ 1018-9068 ] ; 2000.
Descripteurs français
- Pascal (Inist)
- Wicri :
- topic : Enfant.
English descriptors
- KwdEn :
Abstract
Hyperimmunoglobulin M (IgM) syndrome is a rare primary immunodeficiency disorder, and the non-X-linked form of this syndrome is even more infrequent. We report the clinical case of a 6-year-old girl. Her disease began at the age of 1 year when she experienced bronchial obstruction. When she was 3 years old she developed severe recurrent respiratory infections of unusual clinical course. Serum IgM was elevated and the other serum immunoglobulins were absent. Cellular immune response was impaired, with severe depression of delayed hypersensitive cutaneous response and of proliferative response to mitogens. The CD40 ligand expression decreased. Chest CT scan showed areas of lung condensation, bronchial dilatation and signs suggesting interstitial pneumonitis. The latter was confirmed by a biopsy showing a high number of Langerhans' cells and an early-stage fibrosis. She was treated with antibiotics, inhaled bronchodilators and corticosteroids, intravenous immunoglobulin, chloroquine and prednisone. Despite the substitution therapy, her clinical course was slow, with respiratory infections and oxygen dependance. The follow-up thoracoscopic biopsy performed after 18 months of immunosuppressant therapy showed a progression of fibrosis and a decrease in the inflammatory infiltrate.
Affiliations:
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Le document en format XML
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Quezada</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Department of Pediatrics, School of Medicine, University of Chile</s1><s3>CHL</s3><sZ>1 aut.</sZ><sZ>4 aut.</sZ></inist:fA14><country>Chili</country><wicri:noRegion>Department of Pediatrics, School of Medicine, University of Chile</wicri:noRegion></affiliation></author><author><name sortKey="Norambuena, X" sort="Norambuena, X" uniqKey="Norambuena X" first="X." last="Norambuena">X. Norambuena</name><affiliation wicri:level="1"><inist:fA14 i1="02"><s1>Pediatric Service, Hospital Exequiel Gonzalez Cortes</s1><s3>CHL</s3><sZ>2 aut.</sZ><sZ>3 aut.</sZ><sZ>5 aut.</sZ></inist:fA14><country>Chili</country><wicri:noRegion>Pediatric Service, Hospital Exequiel Gonzalez Cortes</wicri:noRegion></affiliation></author><author><name sortKey="Gonzalez, R" sort="Gonzalez, R" uniqKey="Gonzalez R" first="R." last="Gonzalez">R. Gonzalez</name><affiliation wicri:level="1"><inist:fA14 i1="02"><s1>Pediatric Service, Hospital Exequiel Gonzalez Cortes</s1><s3>CHL</s3><sZ>2 aut.</sZ><sZ>3 aut.</sZ><sZ>5 aut.</sZ></inist:fA14><country>Chili</country><wicri:noRegion>Pediatric Service, Hospital Exequiel Gonzalez Cortes</wicri:noRegion></affiliation></author><author><name sortKey="Ramos, S M" sort="Ramos, S M" uniqKey="Ramos S" first="S.-M." last="Ramos">S.-M. Ramos</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Department of Pediatrics, School of Medicine, University of Chile</s1><s3>CHL</s3><sZ>1 aut.</sZ><sZ>4 aut.</sZ></inist:fA14><country>Chili</country><wicri:noRegion>Department of Pediatrics, School of Medicine, University of Chile</wicri:noRegion></affiliation></author><author><name sortKey="Chavez, A" sort="Chavez, A" uniqKey="Chavez A" first="A." last="Chavez">A. Chavez</name><affiliation wicri:level="1"><inist:fA14 i1="02"><s1>Pediatric Service, Hospital Exequiel Gonzalez Cortes</s1><s3>CHL</s3><sZ>2 aut.</sZ><sZ>3 aut.</sZ><sZ>5 aut.</sZ></inist:fA14><country>Chili</country><wicri:noRegion>Pediatric Service, Hospital Exequiel Gonzalez Cortes</wicri:noRegion></affiliation></author><author><name sortKey="Gonzalez, S" sort="Gonzalez, S" uniqKey="Gonzalez S" first="S." last="Gonzalez">S. Gonzalez</name><affiliation wicri:level="1"><inist:fA14 i1="03"><s1>Department of Pathology, Pontifical Catholic University of Chile</s1><s2>Santiago</s2><s3>CHL</s3><sZ>6 aut.</sZ></inist:fA14><country>Chili</country><wicri:noRegion>Santiago</wicri:noRegion></affiliation></author></analytic><series><title level="j" type="main">Journal of investigational allergology & clinical immunology</title><title level="j" type="abbreviated">J. investig. allergol. clin. immunol.</title><idno type="ISSN">1018-9068</idno><imprint><date when="2000">2000</date></imprint></series></biblStruct></sourceDesc><seriesStmt><title level="j" type="main">Journal of investigational allergology & clinical immunology</title><title level="j" type="abbreviated">J. investig. allergol. clin. immunol.</title><idno type="ISSN">1018-9068</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>CD40 ligand</term><term>Case study</term><term>Chemotherapy</term><term>Child</term><term>Chloroquine</term><term>Clinical form</term><term>Complication</term><term>Female</term><term>Hyperimmunoglobulinemia M syndrome</term><term>IgM</term><term>Immunoglobulinemia</term><term>Immunoglobulins</term><term>Immunological investigation</term><term>Immunotherapy</term><term>Interstitial pneumonitis</term><term>Monoclonal immunoglobulinemia</term><term>Treatment</term></keywords><keywords scheme="Pascal" xml:lang="fr"><term>Immunoglobulinémie</term><term>IgM</term><term>Immunoglobulinémie monoclonale</term><term>Enfant</term><term>Femelle</term><term>Etude cas</term><term>Pneumopathie interstitielle</term><term>Complication</term><term>Forme clinique</term><term>Exploration immunologique</term><term>Traitement</term><term>Chloroquine</term><term>Chimiothérapie</term><term>Immunothérapie</term><term>Immunoglobuline</term><term>Ligand CD40</term><term>Hyperimmunoglobulinémie M syndrome</term></keywords><keywords scheme="Wicri" type="topic" xml:lang="fr"><term>Enfant</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Hyperimmunoglobulin M (IgM) syndrome is a rare primary immunodeficiency disorder, and the non-X-linked form of this syndrome is even more infrequent. We report the clinical case of a 6-year-old girl. Her disease began at the age of 1 year when she experienced bronchial obstruction. When she was 3 years old she developed severe recurrent respiratory infections of unusual clinical course. Serum IgM was elevated and the other serum immunoglobulins were absent. Cellular immune response was impaired, with severe depression of delayed hypersensitive cutaneous response and of proliferative response to mitogens. The CD40 ligand expression decreased. Chest CT scan showed areas of lung condensation, bronchial dilatation and signs suggesting interstitial pneumonitis. The latter was confirmed by a biopsy showing a high number of Langerhans' cells and an early-stage fibrosis. She was treated with antibiotics, inhaled bronchodilators and corticosteroids, intravenous immunoglobulin, chloroquine and prednisone. Despite the substitution therapy, her clinical course was slow, with respiratory infections and oxygen dependance. The follow-up thoracoscopic biopsy performed after 18 months of immunosuppressant therapy showed a progression of fibrosis and a decrease in the inflammatory infiltrate.</div></front></TEI><affiliations><list><country><li>Chili</li></country></list><tree><country name="Chili"><noRegion><name sortKey="Quezada, A" sort="Quezada, A" uniqKey="Quezada A" first="A." last="Quezada">A. Quezada</name></noRegion><name sortKey="Chavez, A" sort="Chavez, A" uniqKey="Chavez A" first="A." last="Chavez">A. Chavez</name><name sortKey="Gonzalez, R" sort="Gonzalez, R" uniqKey="Gonzalez R" first="R." last="Gonzalez">R. Gonzalez</name><name sortKey="Gonzalez, S" sort="Gonzalez, S" uniqKey="Gonzalez S" first="S." last="Gonzalez">S. Gonzalez</name><name sortKey="Norambuena, X" sort="Norambuena, X" uniqKey="Norambuena X" first="X." last="Norambuena">X. Norambuena</name><name sortKey="Ramos, S M" sort="Ramos, S M" uniqKey="Ramos S" first="S.-M." last="Ramos">S.-M. 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