[Pulmonary involvement in systemic scleroderma. Part I. Chronic fibrosing interstitial lung disease].
BACKGROUND
Chronic pulmonary interstitial fibrosis is the most frequent respiratory manifestation in systemic sclerosis, occurring in 80% of cases. It remains a severe complication of the disease and is the primary cause of mortality related to respiratory insufficiency in 20 to 60% of cases.


BACKGROUND
The date of onset of interstitial lung disease remains undetermined, and only in rare cases does it reveal the presence of systemic sclerosis. The clinical signs are only observable at a later stage, when at least 50% of the lung parenchyma is affected. The methods of choice adopted for early diagnosis of this disease are high resolution computed tomography and pulmonary functional investigations; they should be carried out during the preliminary investigation and at follow-up once a year. Moreover, high resolution computed tomography also provides prognostic data, for there is a correlation between the type of lesion and its severity as determined by high resolution computed tomography and by histological findings. The value of other methods of investigation, in particular bronchoalveolar lavage, has not yet been clearly established. The association of cyclophosphamide and corticoids is currently being evaluated (indications, administration modalities, duration), and this combination may be the most effective treatment.


CONCLUSIONS
Interstitial lung disease is one of the major causes of morbidity and mortality in systemic sclerosis. Early diagnosis and management of this disease is therefore of utmost importance.
Marie
I
I
Département de médecine interne, centre hospitalier universitaire de Rouen-Boisguillaume, France.
Lévesque
H
H
Dominique
S
S
Hatron
P Y
PY
Michon-Pasturel
U
U
Remy-Jardin
M
M
Courtois
H
H
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D018893:Bronchoalveolar Lavage; D002908:Chronic Disease; D005500:Follow-Up Studies; D006801:Humans; D007091:Image Processing, Computer-Assisted / Q000379:methods; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D011379:Prognosis; D011658:Pulmonary Fibrosis / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology* / Q000503:physiopathology; D012129:Respiratory Function Tests; D012131:Respiratory Insufficiency / Q000209:etiology; D012595:Scleroderma, Systemic / Q000150:complications* / Q000503:physiopathology; D014057:Tomography, X-Ray Computed / Q000379:methods
D000305:Adrenal Cortex Hormones; D007166:Immunosuppressive Agents
Atteinte pulmonaire au cours de la sclérodermie systémique. Partie I. Pneumopathie interstitielle chronique fibrosante.