Idiopathic pulmonary fibrosis (IPF) is a generally fatal disorder with a reported median survival of 3 to 6 yr. This has been based on relatively few studies with diagnoses inconsistently confirmed by adequate lung biopsy. Retrospective analysis of 104 patients with IPF who had open lung biopsy (OLB) at Mayo Medical Center from 1976 to 1985 was performed to establish the overall survival rate, ...

We report a case of platypnea-orthodeoxia that developed in a 64-year-old Japanese woman during an episode of rapidly progressive interstitial pneumonia with polymyositis. Pulmonary infiltrates were predominant in the bilateral lower lobes. The patient was treated successfully with early administration of immunosuppressive therapies and polymyxin B-immobilized fiber column-direct hemoperfusion,...

A 78-year-old man underwent right lower lobectomy for lung cancer. Histopathological examination led to the diagnosis of adenosquamous cell carcinoma. The background lung adjacent to the pleura showed idiopathic pulmonary fibrosis of microscopic usual interstitial pneumonia pattern, although preoperative computed tomography showed no apparent findings of interstitial pneumonia. The patient show...

Pegfilgrastim is a long-acting granulocyte colony-stimulating factor formulation that has been approved for the prevention of febrile neutropenia. We herein report a case of interstitial pneumonia following administration of pegfilgrastim. A 65-year-old man with stage IV small-cell lung cancer was treated with carboplatin and etoposide as third-line chemotherapy. Pegfilgrastim was administered ...

Although the relationship between muscle strength and exercise capacity has been demonstrated in dermatomyositis without lung dysfunction, little is known about the association between exercise capacity and interstitial lung disease in dermatomyositis. Eleven patients with dermatomyositis with interstitial lung disease without the manifestation of muscle weakness and 12 patients with idiopathic...

Amyopathic dermatomyositis (ADM) is a clinical subtype of dermatomyositis, characterized by the lack of motor weakness and the presence of normal muscle enzyme levels. ADM is sometimes accompanied by interstitial pneumonia that shows a rapid progressive course associated with a poor prognosis. We report a 49-year-old patient who presented with nonspecific interstitial pneumonia (NSIP) associate...

Nonspecific interstitial pneumonia has been linked to numerous etiologies including, most recently, haematologic malignancy. We present a 46-year-old woman with recent-onset rheumatologic illness who developed pulmonary symptoms as the presenting feature of biphenotypic acute leukaemia. Chest radiology demonstrated bilateral infiltrates, and lung biopsy revealed nonspecific interstitial pneumon...

BACKGROUND Clinically, amyopathic dermatomyositis is a clinically distinct subgroup of dermatomyositis characterised by unique dermatological manifestations without muscle involvement. Clinically, amyopathic dermatomyositis is frequently associated with interstitial lung disease, which usually has a rapidly progressive, fatal clinical course. Although clinically, amyopathic dermatomyositis-rela...

Background: Vascular remodelling has recently been shown to be a promising pathogenetic indicator in idiopathic interstitial pneumonias (IIPs). Aim: To validate the importance of the collagen/elastic system in vascular remodelling and to study the relationships between the collagen/elastic system, survival and the major histological patterns of IIPs. Methods: Collagen/elastic system fibres were...