Fibrosing alveolitis associated with systemic sclerosis has a better prognosis than lone cryptogenic fibrosing alveolitis.

Abstract

Fibrosing alveolitis associated with systemic sclerosis (FASSc) is considered to be histologically and radiologically indistinguishable from lone cryptogenic fibrosing alveolitis (CFA). To date, the natural history of the two diseases has not been compared directly in large groups of patients followed at a single institution. We evaluated the survival of 205 patients with CFA and 68 patients with FASSc. Deaths during the follow-up period were reported in 142 patients with CFA (70%) and in 11 patients with FASSc (16%). Unadjusted survival from the onset of dyspnea was less in CFA than in FASSc (odds ratio, 3.3; p < 0.001); this difference persisted after adjustment for age of onset of dyspnea and smoking history. Survival from presentation was less in CFA (odds ratio approximately 3.3) after adjustment for age of presentation, smoking history, and initial pulmonary function indices. These differences persisted when analysis was confined to patients with histologic confirmation of fibrosing alveolitis and after adjustment for treatment. In patients undergoing computed tomography (CT) of the lungs, survival was less in CFA after adjustment for extent of abnormal lung on CT and CT pattern of disease (odds ratio, 3.9; p < 0.001). These findings indicate that despite their histologic and radiologic similarities, lone CFA and FASSc have different courses.

The Pulmonary Fibrosis-Associated MUC5B Promoter Polymorphism Does Not Influence the Development of Interstitial Pneumonia in Systemic Sclerosis

“…Finally, we tested for association using a more stringent phenotype defi nition, the most com mon form of IIP. 5 These observations raise questions regarding shared and distinct etiologic and pathologic events underlying the development of lung fi brosis in its idiopathic and SSc-associated forms.…”

Pivotal clinical dilemmas in collagen vascular diseases associated with interstitial lung involvement

“…Therefore, the identification and staging of pulmonary involvement is very important for the management of the disease. For many years, IPF and FASSc were considered to be histologically similar conditions, despite the better prognosis in FASSc [61]. Recent studies in SSc demonstrated that NSIP is the most common histological pattern, followed by UIP in SSc [62][63][64].…”

Update in systemic sclerosis

“…The authors of this present study have published previous articles demonstrating better prognosis in patients with interstitial lung disease secondary to SSc than those with idiopathic pulmonary fibrosis (16,17). More recently, they compared BAL cellularity between the 2 groups and found that patients with cryptogenic or idiopathic fibrosing alveolitis had higher levels of both neutrophils and eosinophils in the BAL fluid, but the eosinophil levels were ultimately found to be linked to more extensive lung disease (18).…”