ESSENTIALS OF DIAGNOSIS
Important to identify specific fibrosing disorders.
Idiopathic disease may require biopsy for diagnosis.
Accurate diagnosis identifies patients most likely to benefit from therapy.
The most common diagnosis among patients with diffuse interstitial lung disease is one of the interstitial pneumonias, including all the entities described in Table 9–17. Historically, a diagnosis of interstitial lung disease was based on clinical and radiographic criteria with only a small number of patients undergoing surgical lung biopsy. When biopsies were obtained, the common element of fibrosis led to the grouping together of several histologic patterns under the category of interstitial pneumonia or idiopathic pulmonary fibrosis (IPF). Distinct histopathologic features are now understood to represent different natural histories and responses to therapy (Table 9–17). Therefore, in the evaluation of patients with diffuse interstitial lung disease, clinicians should attempt to identify specific disorders.
Table Graphic Jump Location Table 9–17.Idiopathic interstitial pneumonias. ||Download (.pdf) Table 9–17. Idiopathic interstitial pneumonias.
|Name and Clinical Presentation ||Histopathology ||Radiographic Pattern ||Response to Therapy and Prognosis |
Usual interstitial pneumonia (UIP)
Age 55–60, slight male predominance. Insidious dry cough and dyspnea lasting months to years. Clubbing present at diagnosis in 25–50%. Diffuse fine late inspiratory crackles on lung auscultation. Restrictive ventilatory defect and reduced diffusing capacity on pulmonary function tests. ANA and RF positive in ~25% in the absence of documented collagen-vascular disease.
|Patchy, temporally and geographically nonuniform distribution of fibrosis, honeycomb change, and normal lung. Type I pneumocytes are lost, and there is proliferation of alveolar type II cells. “Fibroblast foci” of actively proliferating fibroblasts and myofibroblasts. Inflammation is generally mild and consists of small lymphocytes. Intra-alveolar macrophage accumulation is present but is not a prominent feature. ||Diminished lung volume. High-resolution CT scanning shows increased linear or reticular bibasilar and subpleural opacities, with associated honeycombing. Unilateral disease is rare. Minimal ground-glass. Areas of normal lung may be adjacent to areas of advanced fibrosis. ||No randomized study has demonstrated improved survival compared with untreated patients. Inexorably progressive. Median survival ~3 years, depending on stage at presentation. Nintedanib and pirfenidone reduce rate of decline in lung function. Refer early for lung transplantation evaluation. |
Respiratory bronchiolitis–associated interstitial lung disease (RB-ILD)1
Age 40–45. Presentation similar to that of UIP though in younger patients. Similar results on pulmonary function tests, but less severe abnormalities. Patients with respiratory bronchiolitis are invariably heavy smokers.
|Increased numbers of macrophages evenly dispersed within the alveolar spaces. Rare fibroblast foci, little fibrosis, minimal honeycomb change. In RB-ILD the accumulation of macrophages is localized within the peribronchiolar air spaces; in DIP1, it is diffuse. Alveolar architecture is preserved. ||High-resolution CT shows nodular or reticulonodular pattern, more likely to reveal diffuse ground-glass opacities. Honeycombing is rare. May also show upper lobe emphysema. ||Spontaneous remission occurs in up to 20% of patients, so natural history unclear. Smoking cessation is essential. Prognosis clearly better than that of ...|