Idiopathic Interstitial Pneumonia Overview

Idiopathic interstitial pneumonia (IIPs) are interstitial lung disease of unknown etiology, have similar clinical and radiological characteristics and differ mainly by the histopathologic patterns in lung biopsy. They are divided into eight histological subtypes are characterized by varying degrees of inflammation and fibrosis and cause all dyspnea. Diagnosis is based on medical history, physical examination findings, high-resolution CT, pulmonary function tests and lung biopsies. The treatment varies depending on the subtype. The prognosis depends on the subtype, ranging from excellent to almost always lethal.

The eight histological subtypes of IIP are in decreasing order of frequency

Idiopathic interstitial pneumonia (IIPs) are interstitial lung disease of unknown etiology, have similar clinical and radiological characteristics and differ mainly by the histopathologic patterns in lung biopsy. They are divided into eight histological subtypes are characterized by varying degrees of inflammation and fibrosis and cause all dyspnea. Diagnosis is based on medical history, physical examination findings, high-resolution CT, pulmonary function tests and lung biopsies. The treatment varies depending on the subtype. The prognosis depends on the subtype, ranging from excellent to almost always lethal. The eight histological subtypes of IIP in decreasing order of frequency are the diopathische pulmonary fibrosis (histologically commonly referred to as interstitial pneumonia diagnosed Desquamative interstitial pneumonia Non-specific interstitial pneumonia crypto genes organizing pneumonia Respiratory bronchiolitis interstitial lung disease (R picture) Acute interstitial pneumonia lymphoid interstitial pneumonia Idiopathic pleuroparenchymale fibroelastosis These subtypes are characterized by .. different degrees of interstitial inflammation and characterized by fibrosis (1) All cause dyspnea, show diffuse abnormalities in high-resolution CT (HRCT) as well as biopsy inflammation, fibrosis, or both However, the distinction between these subtypes is important because they have different clinical features (see table: main characteristics tegn n appeal of idiopathic interstitial pneumonia), and differently to therapy. Main features of idiopathic interstitial pneumonia disease most commonly affected people symptoms findings in chest x-ray findings Histological pattern Idiopathic at high-resolution CT CT differential diagnosis pulmonary fibrosis More common in men> 50 (> 60% smokers) Chronic (> 12 months) basal predominant reticular abnormality with volume loss and honeycombing Peripheral, subpleural and basal reticular Wa benbildung Traktionsbronchiektase or bronchiectasis Architekturale change asbestosis connective tissue hypersensitivity pneumonitis Common Interstitial pneumonia Desquamative interstitial pneumonia Frequent men aged 30-50 (> 90% smokers) Subacute to chronic (weeks to years) frosted glass opacities in most cases peripheral predominance in the subfields frosted glass opacities Reticular lines hypersensitivity pneumonitis Pneumocystis jirovecii pneumonia Respiratory bronchiolitis interstitial lung disease sarcoidosis Desquamative interstitial pneumonia Non-specific Interstitial Pneumonia More often women, usually aged 40-60 (<40% smokers) Subacute to chronic (months to years) Reticular and milk glass opacities Peripherals, basal, symmetrical reticular opacities Various frosted glass opacities Irregular lines crypto genes organizing pneumonia Desquamative interstitial pneumonia hypersensitivity pneumonitis Idiopathic pulmonary fibrosis nonspecific interstitial pneumonia crypto genes organizing pneumonia mensché n any age, usually at the age of 40-50 (<50% smokers) Subacute (<3 months) Spotty bilateral consolidation peribronchial Spotty consolidation, nodules, or both alveolar Eosinophilic pneumonia lymphoma infection nonspecific interstitial pneumonia Sarcoidosis Vasculitis organizing pneumonia Respiratory bronchiolitis with interstitial lung disease Slightly more men, aged 30 to 50 (> 90% smokers) Subacute (weeks to months) bronchial wall thickening frosted glass opacity diffuse pattern Bronchialwandverdicku ng Centrilobular node Spotty frosted glass opacities Desquamative interstitial pneumonia hypersensitivity pneumonitis Non-specific interstitial pneumonia infection Respiratory bronchiolitis interstitial lung disease Acute Interstitial Pneumonia people of all ages † Abruptly (1-2 weeks) Progressive, diffuse ground glass opacities Diffuse consolidation, ground glass opacities, often with lobular recess Later Traktionsbronchiektasen Acute eosinophilic pneumonia acute Respiratory distress Syndrome (ARDS) hydrostatic edema pneumonia diffuser Alveo larschaden Lymphoid interstitial pneumonia Especially women, of all ages † Chronic (> 12 months) reticular opacities node Diffuse pattern Centrilobular node frosted glass opacities Septal and bronchovascular thickening thin-walled cysts Langerhans cell histiocytosis Carcinomatous metastasis sarcoidosis Lymphoid interstitial pneumonia Idiopathic pleuroparenchymale fibroelastosis No preference during sex, average age of 57 years chronic (> 12 months) Bilateral apical pleural thickening irregular density subpleural consolidation Traktionsbronchiektasen Architekturale change volume loss of the upper lobe hypersensitivity pneumonitis Non-specific Interstitial Pneumonia asbestosis tissue diseases Sarcoidosis Radiation-induced lung disease lung disease through medication Pleuroparenchymale fibroelastosis * Listed in order of decreasing frequency. † No known smoking history Note 1. Travis WD, Costabel U, Hansell DM, et al: An Official American Thoracic Society / European Respiratory Society Statement: Update of the International Multidisciplinary Classification of the Idiopathic Interstitial pneumonias. Am J Respir Crit Care Med 188 (6): 733-748, 2013. Symptoms and signs The symptoms and complaints are not specific in general. Cough and dyspnea on exertion are typical, with varying incidence and progression. Common signs are tachypnea, reduced thoracic breathing expansion bibasilare end-inspiratory dry rales and clubbing. Diagnosis High-resolution CT (HRCT) lung function tests Sometimes surgical lung biopsy IIP should be suspected in any patient with unexplained interstitial lung disease. Physicians, radiologists and pathologists should exchange information in order to determine the diagnosis in individual patients. Possible causes (see Table: Causes of interstitial lung disease) are systematically assessed. For best diagnostic results, the following criteria should be considered in the history: duration of symptoms lung disease in the family, in particular pulmonary fibrosis Former tobacco use (because some diseases especially among current or former smokers occur) Current and of past drug use detailed review of the working and living conditions, including members of the family. A chronological list of the entire period of employment of the patient, including specific duties and known exposures to organic and inorganic substances is to create (causes of interstitial lung disease). Extent, duration and latency of exposure as well as the use of protective devices can be requested. A chest x-ray is performed with abnormal findings in general, but the results are not specific enough to distinguish between the different types. Pulmonary function tests are often performed to assess the severity of physiological impairment, but they do not help distinguish between the different types. Typical results are restrictive physiology with reduced lung volume and diffusion capacity. Hypoxemia often occurs during exercise and may be present even at rest. EineHRCT be distinguished in the air spaces of interstitial spaces, is the most useful method and is always performed. Etiology, extent and spread of the disease can be assessed and underlying or accompanying Erkrankunge be better recognized (eg. As occult mediastinal lymphadenopathy, cancer, emphysema). A HRCT should be performed with the patient in supine and prone positions and include a dynamic expiratory imaging to provide proof of involvement of the small airways. Laboratory tests are performed in patients with clinical features suggestive of connective tissue disease, vasculitis, or environmental influences. These may include tests for antinuclear antibodies, rheumatoid factor, and other spezifischerere serological tests for connective tissue diseases (eg. B., RNP, SSA, SSB, Scl70, Jo-1), “hypersensitivity panel” (a collection of tests for antibodies to common antigens from microbial, fungal and animal sources) and tests for anti-neutrophil cytoplasmic antibodies and antibody Antibasalmembran. A bronchoscopic transbronchial biopsy can be helpful to distinguish certain interstitial lung diseases such as sarcoidosis and hypersensitivity pneumonitis, but it does not give enough tissue to diagnose the IIPs. Bronchoalveolar lavage (BAL) is helpful in some patients for narrowing the differential diagnosis and provides information about disease progression and therapeutic response. The benefit of this investigation to ensure the initial diagnosis and follow-up has so far not been established. A surgical lung biopsy is necessary to confirm the diagnosis when history and HRCT are not diagnostic. For biopsies of several sampling points by a video-assisted thoracoscopic surgery (VATS) are needed. Therapy varies depending on the disease often corticosteroids Sometimes lung transplantation. Treatment depends on the disease (see table: treatment and prognosis of idiopathic interstitial pneumonia *). Smoking cessation is always recommended to avoid the potentially accelerated disease progression and limit respiratory comorbidities. Corticosteroids are typically cryptogenic organizing pneumonia, lymphoid interstitial pneumonia and non-specific interstitial pneumonia recommended, but not in idiopathic pulmonary fibrosis. A lung transplant may be recommended in selected patients with end-stage diseases. Treatment and prognosis of idiopathic interstitial pneumonia * disease treatment prognosis idiopathic pulmonary fibrosis pirfenidone or nintedanib; Lung transplant mortality rate: 50-70% in 5 years Desquamative interstitial pneumonia smoking cessation mortality rate: 5% 5 year nonspecific interstitial pneumonia corticosteroids with or without immunosuppressive therapies (eg, azathioprine, mycophenolate mofetil.) Death rate: highly variable, but usually better than idiopathic pulmonary fibrosis. In pure cellular disease (rare) extremely low Cryptogenic organizing pneumonia corticosteroids Full recovery rate:> 65% relapses: Many death rate: Rare Respiratory bronchiolitis interstitial lung disease smoking cessation mortality rate: Rare Acute Interstitial Pneumonia Supportive treatment mortality rate: 60% at <6 months Lymphoid Interstitial Pneumonia Corticosteroids not well defined Idiopathic pleuroparenchymale fibroelastosis Appropriate treatment is not known; Corticosteroids often a progression of the disease occurs in 60% listed in order of decreasing frequency before *. Conclusion There are eight histological subtypes of idiopathic interstitial pneumonia symptoms, signs and chest X-ray findings are nonspecific. The diagnosis of IIPs is primarily based on the history and on HRCT. If the clinical evaluation and HRCT not lead to the diagnosis, a surgical lung biopsy should be performed. Treatment depends on the subtype.

Health Life Media Team

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