Interstitial lung disease.

Interstitial lung diseases are a group of diffuse parenchymal lung disorders associated with substantial morbidity and mortality. Knowledge achieved in recent years has resulted in the publication of the new classification of idiopathic interstitial pneumonias, according to which there are three groups: major, rare and unclassified. The novelty of the new classification comes from the fact that difficult to classify entities can be treated according to the disease behaviour classification.

The effect of anticoagulant therapy for idiopathic pulmonary fibrosis in real life practice.

Background: IPF is a common form of interstitial lung disease for which there is no effective therapy and usually results in death. Two previous contradictory studies showed anticoagulant therapy  to be associated with both improved and worsened survival, respectively.

Objective: The objective of this retrospective cohort study was to evaluate the effect of anticoagulant therapy on the survival and disease progression of patients with idiopathic pulmonary fibrosis (IPF) in real clinical practice.

Current status of idiopathic nonspecific interstitial pneumonia.

Pulmonary pathologists were aware of cases of idiopathic interstitial pneumonia (IIP) that morphologically did not fit Liebow's classification scheme. These cases were labeled as "cellular interstitial pneumonia" or "chronic interstitial pneumonia not otherwise specified." The term nonspecific interstitial pneumonia (NSIP) was first used in relation to a pattern of lung interstitial inflammation seen in association with human immunodeficiency virus (HIV) infection.

Efficacy of Corticosteroids Alone in the Eradication of Factor VIII Inhibitor in an Old Female with Idiopathic Acquired Haemophilia A: Description of a Case.

Acquired haemophilia A (AHA) is a rare and serious disorder mainly affecting elderly patients. It is caused by the production of autoantibodies directed against coagulation factors; patients present with spontaneous bleeding, potentially fatal, in the absence of familial or personal history. Autoimmune disorders, infections, solid and hematologic tumors, and drugs are predisposing factors, but up to 50 percent of cases remain unexplained.

Pulmonary features of Birt-Hogg-Dubé syndrome: cystic lesions and pulmonary histiocytoma.

Background: to describe clinical, radiologic and pathologic features of lung lesions in Birt-Hogg-Dubè syndrome (BHDS) (MIM 135150).

Method: review of 12 patients of BHDS from 3 unrelated Italian families evaluated at GB Morgagni Hospital, Forlì, from 2005 to 2010.

Results: mean age (±SD) at diagnosis was 44.

Diffuse panbronchiolitis.

Diffuse panbronchiolitis (DPB) is an idiopathic inflammatory disease, well recognised in Japan and principally affecting the respiratory bronchioles, causing a progressive suppurative and severe obstructive respiratory disorder. If left untreated, DPB progresses to bronchiectasis, respiratory failure and death. It was first described in the early 1960s.

Skip lesion of the cecum associated with proctitis: an atypical case of ulcerative colitis.

The authors report the clinical case of a patient who underwent total colectomy for acute ulcerative colitis. The unusual element in this case was the presence of a lesion ('skip lesion'), typical of ulcerative colitis, in the periappendiceal area of the cecum, which was discontinuous to the main site of disease located in the rectum and left colon. The presence of skip lesions, whose clinicopathological relevance is still unknown, would seem to disprove the widely held view that ulcerative colitis involves only the mucous membrane of the large intestine, with inflammatory processes of varying intensity, but without intervening normal areas.

Diffuse panbronchiolitis.

Diffuse panbronchiolitis (DPB) is an idiopathic inflammatory disease, largely restricted to Japan, that is characterized by progressive suppurative and obstructive airway disease, which, if left untreated, progresses to bronchiectasis, respiratory failure, and death. The lesion was first described in the early 1960s. In 1969 the name diffuse panbronchiolitis (DPB) was proposed to distinguish it from chronic bronchitis.