Teaching Case

A 60 years old patient presented in the outpatient department with worsening of a exertional dyspnoe.

A 60-year-old patient presented himself to the outpatient department with exertional dyspnea . His everyday activities were actually limited by worsening dyspnea. The patient had a known systemic sclerosis, which caused only moderate symptoms. Pulmonary function tests showed a slight restrictive ventilation disorder and a severe reduction of diffusion capacity. CT and HR-CT were performed. Multidetector computed tomography (MD-CT) was performed in spiral mode in addition to a high-resolution CT. Imaging showed all signs of pulmonary artery hypertension, including small peripheral pulmonary arteries in contrast to large central arteries, enlarged right ventricle and atrium, pericardial effusion, and horizontally displaced interventricular septum. Vena cava and hepatic veins were enlarged due to right-heart insufficiency. Several enlarged mediastinal lymph nodes were present (Figure 1a). Apical HR-CT images showed only minor pathologic changes: Faint, peripherally located inter- and intralobular septal thickening was present. The same changes were detected in the lower lobes to a much greater degree, causing traction bronchiectasis and ground-glass opacities. The esophagus was dilated and fluid-filled as a consequence of typical organ involvement.

Systemic sclerosis is characterized by induration of the skin, Reynaud's phenomenon (in 80-90%), and musculoskeletal and visceral manifestations. The current patient shows the typical signs of lung involvement in systemic sclerosis. Pulmonary involvement is present in 70-90% of cases (2) and is now the major cause of death (1). Fibrotic changes occur predominantly in the lower lobes and are typically peripherally located. HR-CT findings include 1. ground glass opacification, 2. fine reticular pattern through to honeycombing, 3. lines of various types (septal, subseptal long parenchymal lines) and 4. subpleural and intralobular micronodulation (1). The intralobular thickening may reach the point that it causes ground-glass opacity. Pneumonia is a recognized complication of systemic sclerosis. It may be due to aspiration in cases of severe esophageal dysmotility. However, most authors consider that aspiration does not play a significant role in pathogenesis pulmonary fibrosis. Historically, the fibrosing alveolitis associated with systemic sclerosis was considered the same as cryptogenic fibrosing alveolitis. However, the much better prognosis of fibrosing alveolitis in systemic sclerosis compared to cryptogenic pulmonary fibrosis suggests a different pathogenesis (5). Exertional dyspnea is present in as much as 60% of patients with systemic sclerosis and is due to pulmonary hypertension (3). Pulmonary hypertension in systemic sclerosis, in contrast to pulmonary hypertension in other fibrosing lung diseases, is not strictly coupled to the grade of fibrosis. Both may develop independently. Its presence may be signaled by an isolated decrease in diffusing capacity. Pulmonary lymphadenopathy is present in 32-60% of cases (present case) (4).Findings in systemic sclerosis can be summarized as follows : 1. severe pulmonary hypertension (enlarged pulmonary artery, enlarged right ventricle, horizontal dislocation of the Septum interventriculare, pericardial effusion), 2. signs of basal / peripheral, slowly progressing pulmonary fibrosis, often presenting as ground glass attenuation and intralobular septal thickening, 3. lymphadenopathy in 32-60%, 4. signs of esophageal dysmotility including widening of the esophagus and secondary aspiration pneumonias.