Fig. 18.12 Alveoloseptal amyloidosis in a 58-year-old man who also has biopsy-proved gastric amyloidosis. (a, b) Lung window images of consecutive CT (2.5-mm section thickness) scans obtained at level of liver dome show small nodules along pleura (arrows) and smooth or mildly nodular thickening of interlobular septa (open arrows). Also

note areas of ground-glass opacity. (c, d) CT scans obtained at similar levels to and 40 months after a and b, respectively, demonstrate progressed disease with increased extent of interlobular septal thickening and small nodules in both lungs. Diffusing capacity at this time was 45 % of expected value

treatment, but infliximab can be useful in treating the refractory cases [47]. Remission occurs for more than half of patients within 5 years of diagnosis with few or no consequences. Up to one-third of patients have persistent disease, leading to significant organ impairment.

Pulmonary Alveoloseptal Amyloidosis

Pathology and Pathogenesis

associated systemic amyloidosis. There are two main types of PPA: tracheobronchial amyloidosis and nodular amyloidosis, the deposition of balls of amyloid (usually composed of lambda light chain) within the lung parenchyma. Secondary pulmonary amyloidosis is amyloid deposition in lung parenchyma and around blood vessels in patient with systemic amyloidosis [48].

Symptoms and Signs

Amyloidosis is the extracellular deposition of misfolded proteins in beta-pleated sheets. Primary pulmonary amyloidosis (PPA) is amyloid deposition in the respiratory tract without

Pulmonary alveoloseptal amyloidosis manifests as interstitial lung disease. Patients are often symptomatic with dry cough and slowly progressive dyspnea on exertion. Hemoptysis is rare.