12 Idiopathic interstitial pneumonias Idiopathic pulmonary fibrosis (IPF) = Usual interstitial pneumonia (UIP) Non-specific interstitial pneumonia (NSIP)

†Progressive fibrosis was defined as fibrosis detected by HRCT (i.e. reticular abnormality with traction bronchiectasis with or without honeycombing) that was progressive in terms of worsening of lung function (FVC and/or DL CO) and/or respiratory symptoms and/or chest images. ‡Research funded by Boehringer Ingelheim.

isabela.silva@vch.ca Non-specific interstitial pneumonia (NSIP) is an interstitial lung disease that may be idiopathic or secondary to connective tissue disease, toxins or numerous other causes. Idiopathic NSIP is a rare diagnosis and requires exclusion of these other possible causes. Patients typically present in mid-a … Among the idiopathic interstitial pneumonias, the two entities—idiopathic pulmonary fibrosis (IPF) characterised by the presence of the usual interstitial pneumonia pattern of histopathology (IPF/UIP) and non-specific interstitial pneumonia (NSIP; same nomenclature for the histopathological pattern and idiopathic disease)—have provoked considerable debate. Overall survival for 58 patients with polymyositis or dermatomyositis and associated interstitial lung disease (PM–DM-ILD), compared with 63 historical control patients with biopsy-proven idiopathic pulmonary fibrosis/usual interstitial pneumonia (IPF) and 14 patients with biopsy-proven idiopathic nonspecific interstitial pneumonia (NSIP).

Nsip lung fibrosis

RBILD=Respiratory Bronchiolitis-Interstitial Lung Disease Histologisk NSIP=Non-Specific Interstital Pneumonia Histologisk bild: NSIP Nonspecific interstitial pneumonia (NSIP) is a rare disorder that affects the tissue that surrounds and separates the tiny air sacs of the lungs. These air sacs, called the alveoli, are where the exchange of oxygen and carbon dioxide takes place between the lungs and the bloodstream. Originally conceived as an umbrella term for all otherwise non-classifiable forms of IIP, NSIP has been an entity in its own right since the last consensus conference and is based on the histopathological demonstration of an NSIP pattern, which shows a wide spectrum ranging from predominance of a chronic interstitial inflammation (cellular NSIP) to predominance of interstitial fibrosis (fibrotic NSIP). Idiopathic Non-Specific Interstitial Pneumonia, or “idiopathic NSIP”, is a chronic lung disease in which inflammation and/or scar tissue (“fibrosis”) builds up in the walls of the air sacs of the lungs. Important negative histological findings are the absence of acute lung injury including hyaline membranes, granulomas, organisms or viral inclusions, dominant airways disease or organizing pneumonia, eosinophils and coarse fibrosis.

Patients typically present in mid-a … Based on past difficulties in clinically differentiating patients with idiopathic pulmonary fibrosis (IPF), bronchiolitis obliterans-organizing pneumonia (BOOP), and nonspecific interstitial pneumonia/fibrosis (NSIP), which all manifest clinically as interstitial lung disease, experience with pathologically confirmed examples of the three diseases was reviewed to compare clinical profiles and Vid exacerbation av idiopatisk lungfibros rekommenderas kortikosteroider (T.

gree of inflammation and fibrosis. It is important to differentiate NSIP from other diffuse lung diseases, especially usual interstitial pneumo-nia and hypersensitivity pneumonitis, owing to differences in prog-nosis and treatment. At high-resolution computed tomography, the most common findings suggestive of NSIP are lower lobe peripherally

2 Treatment of Nonspecific Interstitial Pneumonia Many patients with the cellular form of NSIP respond well to oral corticosteroids (usually prednisone), medications that help reduce swelling and inflammation characteristic of the disease. The most common pattern of lung fibrosis in rheumatoid arthritis is UIP followed by NSIP [10, 15].

2003-08-01

Idiopatisk lungfibros (idiopathic pulmonary fibrosis) utgjorde drygt hälften av patienterna med IIP och var därmed den vanligaste undergruppen. Huvudbudskapet i dokumentet från 2002 var att särskilja idiopatisk lungfibros från andra diagnoser inom gruppen IIP eftersom det kliniska förloppet utmärker sig med sämre prognos (2).

However, the subpleura is relatively normal in this case, and this patient’s disease has a central, bronchovascular distribution, which is the hallmark feature of NSIP fibrosis. 2007-01-01 2003-08-01 Common medications associated with lung fibrosis include bleomycin, 89 methotrexate, 90 and even amiodarone, 91 although numerous others can also cause pulmonary fibrosis, and the list of pneumotoxic agents is long. 92 When fibrosis occurs, it may assume a UIP or NSIP pattern, closely mimicking other causes of fibrotic lung disease. It is thus not currently excluded that NSIP is a somewhat different response by the lung to one and the same triggering mechanism. This is also supported by the differentiation already discussed between the cellular and fibrotic NSIP, in which fibrotic NISP may indeed have a clinical course that resembles IPF, while cellular NSIP often responds to steroids and prognostically is much more favourable. Se hela listan på my.clevelandclinic.org Se hela listan på radiopaedia.org Lung biopsies performed on patients with NSIP reveal two different disease patterns – cellular and fibrosing – which are associated with different prognoses. The cellular pattern displays chronic inflammation with minimal fibrosis.
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Two of the most common are IPF and non-specific interstitial Vid exacerbation av idiopatisk lungfibros rekommenderas kortikosteroider (T. Prednisolon initialt i dosen 0,5-1 mg/kg kroppsvikt). Parenteral tillförsel av steroider kan också övervägas (intravenöst metylprednisolon 0,5–1 g dagligen i 3 dagar) Behandling av eventuell specifik orsak till försämring bör givetvis ges. Idiopatisk lungfibros (idiopathic pulmonary fibrosis) utgjorde drygt hälften av patienterna med IIP och var därmed den vanligaste undergruppen. Huvudbudskapet i dokumentet från 2002 var att särskilja idiopatisk lungfibros från andra diagnoser inom gruppen IIP eftersom det kliniska förloppet utmärker sig med sämre prognos (2).

Lung fibrosis: new classifications and therapy.
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Changes are predominantly cellular, with scant fibrosis; Better prognosis; Fibrotic type Changes are predominantly fibrotic, with less cellularity; NSIP is a diagnosis of exclusion; The following features should not be seen Granulomas or giant cells; Bronchiolocentric distribution; Acute lung injury pattern / hyaline membranes

2007-01-01 2003-08-01 Common medications associated with lung fibrosis include bleomycin, 89 methotrexate, 90 and even amiodarone, 91 although numerous others can also cause pulmonary fibrosis, and the list of pneumotoxic agents is long. 92 When fibrosis occurs, it may assume a UIP or NSIP pattern, closely mimicking other causes of fibrotic lung disease.