Idiopathic pulmonary fibrosis and interstitial lung diseases Diagnostic

Diagnostic approach of interstitial lung diseases
The etiological diagnosis of diffuse interstitial lung disease (ILD) is based on the integration of simple information gathered by interview, physical examination and discriminating arguments made by some investigations of the lower respiratory tract (HR-CT scan, pulmonary function tests, biological and bronchoalveolar lavage results).
Obtaining the diagnosis of ILD is an important step but lung biopsy is rarely necessary: the key of ILD etiological diagnosis lies to obtain optimal information while imposing minimal patient aggression.
The diagnostic approach is best when the case is subject to a multidisciplinary discussion between clinician, radiologist and pathologist experienced in the field of ILD.
Idiopathic pulmonary fibrosis Idiopathic pulmonary fibrosis is a chronic disease, which affects more frequently subjects older than 60 years, males, and smokers or ex-smokers. The diagnosis is based on a pattern of usual interstitial pneumonia at high resolution computed tomography of the chest and/or at the videoassisted thoracic surgical lung biopsy, and by multidisciplinary discussion in a specialized center, after ruling out possible causes and specific contexts (as connective tissue disease).
The disease progresses inexorably to chronic respiratory insufficiency, occasionally with episodes of acute exacerbation, and death after a median of 3 years. Treatment with pirfenidone in patients with mild to moderate disease slows the progression of disease, and reduces the risk of death at one year, with skin and gastro-intestinal adverse events. Drug therapy should be part of a global care management. Several approaches are considered for an earlier diagnosis and treatment.
Interstitial lung disease in connective tissue disorders: After immunosuppressive-induced infections, interstitial lung disease (ILD) is one of the most serious pulmonary complications associated with connective tissue diseases (CTD). Although it is common for ILD to be diagnosed concurrent with or after CTD, some patients will present with ILD years prior to receiving a diagnosis of CTD. The clinical approach involves an examination of the extrathoracic symptoms (suggestive of CTD) and the evaluation of respiratory disability. Nonspecific interstitial pneumonia is the most common histological finding in patients with CTD. The management of patients with CTD-associated ILD is optimized by multidisciplinary collaboration. ILD-CTD are treated through anti-inflammatory medication, immunosuppressants and biological agents.
Granulomatous interstitial lung diseases
Sarcoidosis is sometimes severe and, in this setting, some investigations like thoracic computed tomography and pulmonaruy function tests constitute an angular stone. In 25% of cases, the presentation is not typical and diagnosis may be difficult. Some lung granulomatosis may share a very similar presentation with sarcoidosis according to clinic, imaging, serum biology, broncho-alveolar lavage and pathology (berylliosis, immuno-deficiency and druginduced lung granulomatosis). Eventually, lung Langerhans histiocytosis is a very rare disease observed in young adults with heavy smoking habits and thoracic CT is the crucial investigation to reach diagnosis in 80% of cases.

[@laal5400]

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Un grand merci pour votre travail .

[@gabrielkendy3978]

bon travail