Interstitial lung disease and adult-onset still's disease

Abstract

Adult-onset Still's disease is an uncommon rheumatological syndrome with a diversity of signs and symptoms. Pulmonary manifestations described are pleuritis and usually transient radiologic infiltrations. The patient presented in this case report had biopsy-proven lung fibrosis when adult-onset Still's disease was diagnosed. Three years after diagnosis, the patient developed clinical signs of the interstitial lung disorder. Radiological and histological progression was observed. Other causes of interstitial lung disorders were excluded. Clinicians should be aware that interstitial lung disease can be a complication of adult-onset Still's disease and can compromise the clinical status of the patient.

Pulmonary Hypertension and Other Potentially Fatal Pulmonary Complications in Systemic Juvenile Idiopathic Arthritis

“…Although isolated case reports of PAH, AP, and ILD in systemic JIA and adult-onset Still's disease have been pub-lished (22)(23)(24)(25)(26)(27)29,30), the findings in this study indicate that these complications may occur more commonly than previously suspected and are fatal in the majority of patients. PAH, ILD, and AP are all processes in which inflammation likely has an important role; for example, pathologic PAH specimens show an increased inflammatory perivascular infiltrate, including macrophages, dendritic cells, lymphocytes, and mast cells (31)(32)(33).…”

“…Although pleuritis, along with pericarditis, is a commonly recognized feature of systemic JIA (11,21), other pulmonary complications, such as PAH, interstitial lung disease (ILD), and alveolar proteinosis (AP) or lipoid pneumonia, are extremely rare. Prior to 2008, only scattered single case reports of PAH, AP, ILD, and illnesses that may represent one of these conditions in systemic JIA and Still's disease existed in the literature (22)(23)(24)(25)(26)(27), so the number of cases being reported to the Listserv represented an increase. Because IL-1 inhibitors became much more commonly used in systemic JIA in the mid-2000s, there was concern about a possible association between exposure to biologic medications such as IL-1 inhibitors and the development of pulmonary complications in some patients (16,17).…”

Interstitial Lung Disease: A Rare Association with Adult-Onset Still’s Disease

“…21 Pericardial effusions and myocarditis are reported cardiac complications. 22 Pleural effusions, fibrosis, and seldom acute respiratory distress syndrome (ARDS) constitute pulmonary involvement. 23 Pulmonary associations with AOSD are rare, accounting for 12-35% for pleuritis and 0-27% for interstitial pneumonia.…”

“…21 The restrictive ventilatory pattern in AOSD is said to be responsive to combined steroid and aspirin therapy. 22 Histological lung features in AOSD include nonspecific interstitial fibrosis, acute alveolitis with thickened alveolar walls, hyperplasia of Type II pneumocytes, hyaline membrane formation, and l ymphocytic infiltration. 23 There are many diagnostic criteria used for the definitive diagnosis of AOSD, with Yamaguchi's being most widely used.…”

A case of adult‐onset Still’s disease complicated by hemophagocytic syndrome and interstitial pneumonia with pneumomediastinum/recurrent pneumothorax

“…However, pneumomediastinum and recurrent pneumothorax occurred. IP has been reported in AOSD, 7,8 but IP with pneumomediastinum and/or pneumothorax has not been previously reported. There is a report of a large series of 62 patients with connective tissue disease complicated by pneumomediastinum, and almost all (79.9%) had polymyositis (PM)/dermatomyositis (DM).…”