Presentation
UCTD was considered present when serum autoantibodies were present and symptoms or signs suggested CTD. The relationship between UCTD and NSIP histology was evaluated. [ncbi.nlm.nih.gov]
Entire Body System
- Inflammation
Our findings provide further evidence of the importance of inflammation in the pathophysiology of IIPs. [ncbi.nlm.nih.gov]
Fibrosis and inflammation Basal, peripheral GGO Basal, peripheral GGO Reticular Reticular Collagen vascular Collagen vascular Drug reaction Drug reaction DIP DIP Chronic hypersensitivity Chronic hypersensitivity Macrophages in alveoli Macrophages in [learningradiology.com]
[…] present Ground glass opacities focal but not widespread Classic cases can be diagnosed by xray and do not need biopsy confirmation Histopathologic features Heterogeneous pattern of alternating honeycombing and normal lung with intermediate zones of inflammation [surgpathcriteria.stanford.edu]
Microscopic criteria in diagnosis of these entities include the pattern and microanatomic distribution of inflammation, fibroblast proliferation, collagen deposition, and architectural remodeling. [doi.org]
- Pathologist
Three clinicians, two radiologists, and two pathologists reviewed 58 consecutive cases of suspected idiopathic interstitial pneumonia. [ncbi.nlm.nih.gov]
- Lymphadenopathy
Lymphadenopathy in left hilus, right hilus and paratracheal (1-2-3 sign). Often with calcifications. Uncommon findings: Conglomerate masses in a perihilar location. [radiologyassistant.nl]
Examination reveals a well appearing child with a maculopapular rash and posterior cervical lymphadenopathy. Which of the following is the most likely causative organism for this disease? A. Measles virus B. Mumps virus C. Parvovirus B19 D. [roshreview.com]
The likelihood of lymphadenopathy increases with greater extent of disease. 30,31 The prevalence of mediastinal lymphadenopathy in NSIP is similar to that in IPF. 30,31 Acute Exacerbation of Nonspecific Interstitial Pneumonia Similar to patients with [journals.lww.com]
Franquet T, Gimenez A, Alegret X et al (1998) Mediastinal lymphadenopathy in cryptogenic fibrosing alveolitis: the effect of steroid therapy on the prevalence of nodal enlargement. Clin Radiol 53:435–438 CrossRef PubMed Google Scholar 37. [link.springer.com]
The presence of extensive ground-glass opacities, nodules, upper lobe or mid-zone predominance of findings, and significant hilar or mediastinal lymphadenopathy should question the radiographic diagnosis of IPF. [dx.doi.org]
- Asymptomatic
Patients with early ILD changes may be asymptomatic. Features of ILD overlap among systemic diseases and with idiopathic variety. High-resolution computed tomography plays a central role in diagnosing ILDs. Imaging features are often nonspecific. [experts.umich.edu]
Spaggiari E, Zompatori M, Verduri A et al (2005) Early smoking-induced lung lesions in asymptomatic subjects. Correlations between high resolution dynamic CT and pulmonary function testing. Radiol Med 109:27–39 PubMed Google Scholar 18. [link.springer.com]
Clinical courses of asymptomatic cases with idiopathic pulmonary fibrosis and a histology of usual interstitial pneumonia [abstract]. Eur Respir J 1998 ;11: 131s. Google Scholar 24. [dx.doi.org]
Clinical courses of asymptomatic cases with idiopathic pulmonary fibrosis and a histology of usual interstitial pneumonia [abstract]. Eur Respir J 1998; 11 :131s. [ Google Scholar ] 24. [ncbi.nlm.nih.gov]
All smokers have various degrees of respiratory bronchiolitis, but it is usually asymptomatic. [radiologyassistant.nl]
- Hodgkin Lymphoma
A very important differential diagnosis is from Hodgkin’s lymphoma [ 99 ]. Pleuroparenchymal fibroelastosis (PPFE) Idiopathic PPFE has been recently included in the ATS/ERS IIPs classification [ 6 ] as a rare IIP. [link.springer.com]
Respiratoric
- Respiratory Distress
Distress Syndrome Network: Ventilation with lower tidal volumes as compared with traditional tidal volumes for acute lung injury and the acute respiratory distress syndrome. [books.google.com]
distress syndrome Hydrostatic edema Pneumonia Diffuse alveolar damage Lymphocytic interstitial pneumonia Mostly women, of any age † Chronic ( > 12 mo) Reticular opacities Nodules Diffuse pattern Centrilobular nodules Ground-glass opacification Septal [merckmanuals.com]
distress syndrome Hydrostatic edema Pneumonia Diffuse alveolar damage Lymphocytic interstitial pneumonia Mostly women, of any age† Chronic (> 12 months) Reticular opacities Nodules Diffuse pattern Centrilobular nodules Ground-glass opacification Septal [msdmanuals.com]
- Respiratory Insufficiency
Inclusion criteria include: Clinical criteria: chronic respiratory insufficiency manifestations including dyspnea/tachypnea, cough, and cyanosis during exercise or at rest Radiological criteria: characteristic chest High-Resolution Computed Tomography [epidemiologie-france.aviesan.fr]
In a second patient (B), with respiratory insufficiency and severe reduction of DLCO (39 %), the UIP pattern was again diagnosed by CT examination. C and D show diffuse honeycombing, which massively involves the basal regions of lower lobes. [link.springer.com]
- Exertional Dyspnea
The history and results of physical and laboratory evaluations have little diagnostic accuracy for IPF. 48 In all chronic forms of idiopathic interstitial pneumonia, patients usually present with progressive dyspnea on exertion and dry cough. [archinte.ama-assn.org]
Most patients present with a gradual onset (often >6 mo) of exertional dyspnea and/or a nonproductive cough. Approximately 5% of patients have no presenting symptoms when idiopathic pulmonary fibrosis is serendipitously diagnosed. [emedicine.medscape.com]
Gastrointestinal
- Nausea
[…] for anyone who develops severe signs and symptoms of hyponatremia, such as nausea and vomiting, confusion, seizures, or lost consciousness. [mayoclinic.org]
Moderate cases can cause nausea and headaches. In severe cases, hyposmolality can cause seizures and coma and may be life threatening, but such cases are rare. [kidshealth.org]
Most Common Adverse Reactions The most common adverse reactions occurring more frequently (>3%) on Adempas than placebo were headache (27% vs 18%), dyspepsia/gastritis (21% vs. 8%), dizziness (20% vs 13%), nausea (14% vs 11%), diarrhea (12% vs 8%), hypotension [prnewswire.com]
- Vomiting
[…] for anyone who develops severe signs and symptoms of hyponatremia, such as nausea and vomiting, confusion, seizures, or lost consciousness. [mayoclinic.org]
Reactions The most common adverse reactions occurring more frequently (>3%) on Adempas than placebo were headache (27% vs 18%), dyspepsia/gastritis (21% vs. 8%), dizziness (20% vs 13%), nausea (14% vs 11%), diarrhea (12% vs 8%), hypotension (10% vs 4%), vomiting [prnewswire.com]
Workup
Studies have shown that cryobiopsy can provide a definitive diagnosis in many patients undergoing workup for IIP, eliminating the need for surgical lung biopsy. [clinicaladvisor.com]
It is possible that bronchiolocentric interstitial pneumonia represents a form of chronic hypersensitivity pneumonitis for which the inhalational stimulus has not been identified despite our extensive workup for this condition. [nature.com]
See Workup for more detail. Management The optimal medical therapy for the treatment of idiopathic pulmonary fibrosis has yet to be identified. [emedicine.medscape.com]
Treatment
Although pulmonary (67)Ga uptake significantly decreased after treatment (P = 0.001), there was no correlation between either initial (67)Ga uptake or change in (67)Ga uptake on treatment and 1-y prognosis. [ncbi.nlm.nih.gov]
steroids Associations: Sjögren, AIDS Treatment: smoking cessation Mortality 30% at 10 yearsTreatment: ventilation/steroids Multiple basal predominant Treatment: steroids Upper lung predominant faint Treatment: smoking cessationDiffuse symmetric basal [slideshare.net]
Novel treatments (pirfenidone and nintedanib) are available but choice of treatment, duration and impact on a real life scale are unknown. 6 Classification of IIPs (ATS/ERS 2013) 3 References: Travis WD, Costabel U, Hansell DM, King TE, Lynch DA. [swissiip.ch]
Females of reproductive potential: Exclude pregnancy before the start of treatment, monthly during treatment, and 1 month after stopping treatment. [prnewswire.com]
Prognosis
In patients with IPF or fibrotic NSIP, pulmonary (67)Ga scintigraphy may be useful for predicting response to therapy and prognosis. [ncbi.nlm.nih.gov]
Predicting prognosis in the individual patient is challenging but various clinical and radiologic variables have been identified. [doi.org]
Etiology
As the name implies, the etiology is unknown. In the more severe forms of IIP, such as idiopathic pulmonary fibrosis and acute interstitial pneumonia, no effective therapies have been identified. [ncbi.nlm.nih.gov]
Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology. [icd10coded.com]
[…] such as connective tissue diseases and hypersensitivity pneumonia IPF was previously called chronic interstitial pneumonitis or cryptogenic fibrosing alveolitis Sites Bottom of lower lobes of bilateral (or "bibasilar") lung Pathophysiology / etiology [pathologyoutlines.com]
Epidemiology
Jump to: navigation, search Idiopathic Interstitial Pneumonia Microchapters Home Patient Information Overview Historical Perspective Classification Pathophysiology Causes Differentiating Idiopathic interstitial pneumonia from other Diseases Epidemiology [wikidoc.org]
SETTING: Although the incidence and prevalence of idiopathic interstitial pneumonia (IIP) and idiopathic pulmonary fibrosis (IPF) have been assessed in Western countries, their epidemiology has not been analysed in Asian countries, including the Republic [ncbi.nlm.nih.gov]
COP: Epidemiology and outcomes The initial name of COP was bronchiolitis obliterans with organizing pneumonia (“BOOP”). [clinicaladvisor.com]
Idiopathic Interstitial Pneumonia Aka: Idiopathic Pulmonary Fibrosis, Usual Interstitial Pneumonia, Desquamative Interstitial Pneumonia, Respiratory Bronchiolitis, RB-ILD, Acute Interstitial Pneumonia, Hamman-Rich Disease, Nonspecific Interstitial Pneumonia Epidemiology [66.199.228.237]
Contents Complications and Discontinuation 339 Oxygen Therapy and Toxicity 345 ProtocolDriven Care in Respiratory Therapy 351 Epidemiology Natural History 357 Prophylaxis 364 Chronic Thromboembolic Pulmonary Hypertension 375 Unusual Forms of Embolism [books.google.com]
Pathophysiology
New insight into the pathophysiology of idiopathic pulmonary fibrosis suggests a distinctly fibroproliferative process, and antifibrotic therapies show promise. [ncbi.nlm.nih.gov]
Jump to: navigation, search Idiopathic Interstitial Pneumonia Microchapters Home Patient Information Overview Historical Perspective Classification Pathophysiology Causes Differentiating Idiopathic interstitial pneumonia from other Diseases Epidemiology [wikidoc.org]
Prevention
Since IP itself is likely a risk factor for PP, prevention of BPF is important for patients with IP, as it can lead to PP. [ncbi.nlm.nih.gov]
Prevention of bacterial endocarditis. Page 303 - American Thoracic Society. Standards for the diagnosis and care of patients with chronic obstructive pulmonary disease (COPD) and asthma. [books.google.com]
Prevention The following measures may help you prevent hyponatremia: Treat associated conditions. Getting treatment for conditions that contribute to hyponatremia, such as adrenal gland insufficiency, can help prevent low blood sodium. [mayoclinic.org]
Can interstitial lung disease be prevented? Interstitial lung disease can be prevented only to the extent that its individual causes may be prevented. [medicinenet.com]