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Titolo:
Development of progressive pulmonary interstitial and intra-alveolar cholesterol granulomas (PICG) associated with therapy-resistant chronic systemicjuvenile arthritis (CJA)
Autore:
Schultz, R; Mattila, J; Gappa, M; Verronen, P;
Indirizzi:
Tampere Univ Hosp, Dept Pediat, Tampere 33521, Finland Tampere Univ Hosp Tampere Finland 33521 t Pediat, Tampere 33521, Finland Tampere Univ Hosp, Dept Pathol, Tampere 33521, Finland Tampere Univ Hosp Tampere Finland 33521 t Pathol, Tampere 33521, Finland Univ Hannover, Childrens Hosp, Dept Pediat Pulmonol & Neonatol, Hannover, Germany Univ Hannover Hannover Germany t Pulmonol & Neonatol, Hannover, Germany
Titolo Testata:
PEDIATRIC PULMONOLOGY
fascicolo: 5, volume: 32, anno: 2001,
pagine: 397 - 402
SICI:
8755-6863(200111)32:5<397:DOPPIA>2.0.ZU;2-7
Fonte:
ISI
Lingua:
ENG
Soggetto:
NECROSIS-FACTOR-ALPHA; RHEUMATOID-ARTHRITIS; LIPOID PNEUMONIA; LUNG-DISEASE; CHLORAMBUCIL; METHOTREXATE; ACTIVATION;
Keywords:
cholesterol pneumonia; lipoid pneumonia; pulmonary fibrosis; juvenile chronic arthritis; tumor necrosis factor alpha antagonist; etanercept;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Citazioni:
22
Recensione:
Indirizzi per estratti:
Indirizzo: Schultz, R Tampere Univ Hosp, Dept Pediat, POB 2000, Tampere 33521, Finland Tampere Univ Hosp POB 2000 Tampere Finland 33521 3521, Finland
Citazione:
R. Schultz et al., "Development of progressive pulmonary interstitial and intra-alveolar cholesterol granulomas (PICG) associated with therapy-resistant chronic systemicjuvenile arthritis (CJA)", PEDIAT PULM, 32(5), 2001, pp. 397-402

Abstract

A girl aged 5 years with therapy-resistant chronic systemic juvenile arthritis (CJA) developed progressive fibrosing lung disease. Histology of an open lung biopsy revealed pulmonary interstitial and intra-alveolar cholesterol granulomas (PICG). Since treatment with steroids and immunosupressive drugs did not prevent progression of lung fibrosis, an experimental treatment with a tumor necrosis factor alpha (TNF alpha) antagonist etanercept was started. Although development of chronic changes in the lung parenchyma could not be prevented, this treatment brought considerable relief and markedly improved the child'sphysical capacity. By ruling out other causes for development of PICG, we concluded that the primary disease had caused the development of cholesterol granulomata by macrophage activation. We suggest, therefore, that a trial with etanercept in children with otherwise therapy-resistant CJA should be considered, especially if pulmonary complications have developed. (C) 2001 Wiley-Liss, Inc.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 28/03/20 alle ore 23:15:00