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Usual Interstitial Pneumonia Treatment Guidelines

Initial Tests In Suspected Sarcoidosis

Usual Interstitial Pneumonia (UIP)

The diagnosis rests on the correct clinical setting, typical chest radiographic or HRCT appearances and a biopsy showing non-caseating granulomas. A number of other tests may be decisive in shifting diagnostic probabilities in marginal cases or in strengthening the clinical probability such that tissue biopsy is unnecessary. Depending on the local prevalence, tuberculosis may be the most likely alternative diagnosis at presentation and patients should have a tuberculin test and appropriate mycobacterial cultures of any biopsied tissues. A peripheral blood lymphopenia is often present in the active phase of sarcoidosis but is not diagnostic. The serum ACE is considered by many to be a useful test but, with a low sensitivity and poor specificity, it cannot be used as part of the diagnostic algorithm. It does not correlate with chest radiographic stage of disease in sarcoidosis, although it does correlate with the extent of nodules and consolidation on the HRCT scan. However, the general consensus is that serum ACE does not add to the predictive value of serial lung function testing and imaging in disease management. The test result needs to be considered in the light of the various polymorphisms of the ACE gene and the resultant variation in peripheral blood ACE levels. The cerebrospinal fluid ACE level cannot be used to discriminate between neurosarcoidosis and other disorders.

What Are The Symptoms Of Nonspecific Interstitial Pneumonia

A person with nonspecific interstitial pneumonia may have these symptoms:

  • A dry cough.
  • Shortness of breath, which may occur after effort or become worse over time.
  • Difficult or labored breathing.
  • Fatigue.
  • Clubbing, or enlargement of the fingertips at the base of the nails. Clubbing may be present due to a lack of oxygen in the blood. Generally, this occurs in only about 10% of people with NSIP.

Ph Associated With Other Ctds

The true incidence of PH in most CTDs is not known. The available data are largely retrospective in nature and the methods used to measure PAP and to define PH vary. However, PH is a well recognised complication of SLE and may be present in 614% of patients. In a large study of PH in patients with CTD, 8% of 83 subjects with mixed connective tissue disease had PH based on TTE. PH in association with Sjogrens syndrome, PM/DM and RA has only been reported rarely.

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Severe Acute Respiratory Syndrome

An acute viral respiratory tract infection caused by SARS-CoV. World Health Organization. WHO guidelines for the global surveillance of severe acute respiratory syndrome : updated recommendations. Oct 2004 . It was first identified in the Guangdong province of Southern China in 2002. The epidemic affected 26 countries and resulted in more than 8000 cases and 774 deaths in 2003. There have been no reported cases since 2004. The case fatality rate is approximately 10%. World Health Organization. Summary of probable SARS cases with onset of illness from 1 November 2002 to 31 July 2003. Jul 2015 .

What Is Interstitial Lung Disease

Histopathological criteria for usual interstitial pneumonia (UIP ...

Interstitial lung disease is a group of many lung conditions. All interstitial lung diseases affect the interstitium, a part of your lungs.

The interstitium is a lace-like network of tissue that goes throughout both lungs. It supports your lungs’ tiny air sacs, called alveoli. Normally, the interstitium is so thin that it doesnât show up on X-rays or CT scans.

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What Is Nonspecific Interstitial Pneumonia

Nonspecific interstitial pneumonia 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.

With interstitial pneumonia the mesh-like walls of the alveoli become inflamed. The pleura might become inflamed as well. Over time, this inflammation can lead to permanent scarring of the lungs.

NSIP can be found in a number of different diseases, including connective tissue disorders , reactions to certain medications, HIV, as well as other conditions. Some patients also have idiopathic NSIP, which means that the specific cause of the lung disease is unknown.

Diagnosis Of Ph In Ild

The clinical symptoms and signs of PH characteristically appear late in the course of ILD and the symptoms will often be masked by the underlying pulmonary disorder.

The gold standard for measuring PAP and thereby diagnosing PH is right heart catheter. This invasive test carries with it a small but inherent risk to the patient. There is therefore a need for simple and reliable non-invasive indicators of PH.

Lung function testing

A reduced Tlco is of only limited value in predicting PH in the presence of ILD. In IPF the prevalence of PH has been shown to be significantly higher in subjects with Tlco< 40% predicted and resting oxygen saturation < 88%. In patients with SSc without evidence of concomitant lung disease, Tlco< 50% predicted or a declining Tlco on serial testing does predict for the presence of PAH. The role of submaximal exercise testing in detecting PH in ILD is unknown.

Transthoracic Doppler echocardiography

In summary, TTE is a non-invasive, cost-effective and widely available investigation which is suitable for screening patients for PH. The ideal threshold for PAP determined by TTE above which one should proceed to right heart catheterisation is uncertain, and if PH is clinically suspected, right heart catheter should always be considered.

Brain natriuretic peptide

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Staging The Severity And Progression Of Disease

Evaluation of the chest radiograph provides, at best, a crude approximation of disease extent in ILD. Pulmonary function tests usually reflect the severity of disease more accurately than chest radiography. However, the wide variation in the normal range of pulmonary function tests is a major constraint in the staging of mild disease. For example, apparently minor impairment in levels of forced vital capacity to 75% of predicted can represent anything from a 5% to 45% decline from premorbid values. Moreover, pulmonary function tests are readily confounded by concurrent disease processes. The most widely studied example is the frequent admixture of emphysema and fibrosis seen in up to 40% of patients with IPF in some series, and producing a spurious preservation in spirometric and plethysmographic lung volumes but a disproportionate reduction in gas transfer. In the connective tissue diseases a variety of abnormal processes, including interstitial, pulmonary vascular, pleural and muscle disease, commonly coexist but, without an independent means of evaluating the morphological extent of each, clinicians face difficulty in understanding and deconstructing patterns of lung function impairment.

Hrct In Ild: Conclusions

IPF Animated Video: Diagnosis of Interstitial Lung Disease

HRCT is significantly superior to chest radiography in identifying and determining the correct diagnosis of ILD and the optimal site of biopsy for patients requiring a tissue diagnosis.

The combined information from clinical, laboratory and HRCT findings allows a correct diagnosis to be made in the majority of patients with ILD.

The combination of clinical features and HRCT appearances typical of IPF obviates the need for histopathological confirmation of the diagnosis.

A standard HRCT protocol is optimal in most patients with ILD and the need for extra techniques or volumetric HRCT must be carefully justified, given the extra radiation burden to the patient.

HRCT interpretation is a specialised task and requires an understanding of the clinical and pathological aspects of ILD.

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Prognosis And Predictive Factors

The prognosis of interstitial pneumonia is grim, with short-term mortality rates in excess of 50% in most reported series. In most patients, UIP follows a progressive course, with median survivals from the time of diagnosis of about 3 years.

No single histologic finding consistently predicts prognosis in individual patients with UIP. Patients with more extensive fibroblast foci have experienced shorter mean survival in some studies while other investigators have failed to demonstrate the same relationship to survival.

The natural history of idiopathic pulmonary fibrosis has been known as a steady decline in lung function over time without a response to medical therapy. However, the clinical course of IPF is not always predictable despite its generally poor prognosis.

Some common genetic variants have also been associated with distinct clinical phenotypes. Clinical studies should be designed controlling for the genetic backgrounds of subjects, since clinical outcomes and therapeutic responses may differ by genotypes. Further understanding of these differences will allow the development of personalized approaches to IPF management.

Summary Of Chest Radiography And Hrct Recommendations

  • Radiologists with an interest in thoracic imaging and respiratory physicians should meet regularly to evaluate imaging in patients with ILD.

  • In patients for whom the diagnosis is uncertain after chest radiography and clinical assessment, HRCT scanning is the next investigation of choice.

  • HRCT is valuable in detecting ILD in patients with a normal chest radiograph.

  • In the appropriate clinical setting, appearances on the HRCT scan may be sufficiently characteristic to preclude the need for BAL or lung biopsy and histopathological confirmation.

  • Radiologists involved with determining the protocol and interpretation of HRCT scans should have expertise in the technique, be responsible for quality assurance and ensure that an appropriate radiation dose protocol is used. At least one radiologist in any department should have a declared interest and be trained in chest radiology and HRCT.

  • Consideration should be given to establishing a reference panel of radiologists with particular expertise in HRCT.

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Summary Of Recommendations For Bal And Tblb In Ild

  • BAL or TBLB, when required, should be performed before the initiation of treatment.

  • BAL should be considered in all patients with suspected infection, malignancy and some rare ILDs. In such cases, BAL may be diagnostic.

  • BAL is not required as a diagnostic tool in patients with clinical features and HRCT appearances typical of IPF.

  • In patients for whom the diagnosis is uncertain after clinical assessment and HRCT scanning, typical BAL cellular profiles may allow a diagnosis of HP or sarcoidosis to be made with greater confidence.

  • In cases in which the diagnosis is uncertain and BAL is considered, the procedure should be performed in a regional centre with technical expertise in the procedure and the analysis of the BAL samples.

  • BAL should be performed in all patients undergoing TBLB.

  • TBLB is the initial procedure of choice in those patients likely to have ILDs in which small samples may be diagnostic, particularly if the disease has a tendency for bronchocentric involvement. HRCT should be used to guide the biopsy site.

  • Four to six TBLB specimens should be taken.

  • In suspected sarcoidosis, endobronchial biopsy samples in addition to TBLB are recommended since they are frequently positive, are associated with low morbidity and increase the diagnostic yield.

  • TBLB is not recommended as the initial biopsy option in cases of suspected IPF and is unreliable in the diagnosis of rare lung disease .

Histopathology In The Uip Group

High resolution computed tomography images demonstrating usual ...

At low magnification, the lesions varied in severity and were distributed erratically. Chronic and acute lesions of interstitial inflammation, fibrosis, and honeycombing were interspersed among the normal lung tissue, and were primarily within the subpleural lung parenchyma. The interstitial inflammation was typically patchy with alveolar septum infiltrates comprising leukomonocytes and plasmacytes accompanied by type II alveolar cell proliferation. Diffuse hyperplastic fibrous tissue with collagen deposition formed the alveolar structure. In the areas of inflammation, fibrosis, and honeycomb changes, foci in a light-blue myxoid stroma background were observed. These foci comprised proliferative fibroblast and myoblast cells were identified as myofibroblast cell foci. The honeycombed lung formed by a cystic fiber chamber was often covered by bronchial epithelial cells and contained mucus. In the fibrotic and honeycomb areas, smooth muscle proliferation was observed, which was patchy and at times with myogelosis. In two cases, mixed diffuse alveolar damage, cell proliferation, and the loss of the alveolar epithelial cells were also present. The lung interstitium also showed fibroblast proliferation and a macrophagocyte mass along with serous effusion from the alveolar space. The incidences of each particular lesion in both patient groups are summarized in .

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Management Of Specific Ild Entities On The Icu

As described, the three most frequent ILD diagnoses are AIP, acute exacerbation of IPF and fulminant COP. Management of coexisting infection and general supportive measures should be guided by local practice and national guidelines in close liaison with microbiological advice. Specific therapies for ILD in this setting have generally consisted of high-dose intravenous corticosteroid therapy and other forms of immunosuppression. However, in the absence of any controlled data on treatment of rapidly progressive ILD, the guidance is largely based on anecdotal experience.

Finally there are three clinical scenarios that are particularly prone to suboptimal management as a consequence of misdiagnosis:

Appendix : Aide Memoire For The First Clinic Visit In Suspected Ild

In addition to routine respiratory history and examination:

  • History of acid reflux, symptoms suggestive of connective tissue disease including Raynauds phenomenon.

  • Detailed occupational history.

  • Exposure to birds and other potential antigens.

  • Current and previous drugs

Investigations for most patients with suspected ILD:

  • Full blood count, urea and electrolytes, calcium, lung function tests, ESR, CRP, rheumatoid factor, ANA.

  • Oxygen saturation at rest.

  • Spirometry, lung volumes and gas transfer.

  • ECG.

Additional tests in selected patients:

  • Suspected sarcoidosis: serum ACE

  • Suspected hypersensitivity pneumonitis: precipitating antibody to suspected antigen

  • Suspected vasculitis: ANCA

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Summary Of Recommendations For The Care Pathway And General Management Strategies For Ild

  • All patients with ILD should have access to a multidisciplinary team based in a regional centre with expertise in ILD.

  • Referral to a regional ILD clinic should be made if there are perceived difficulties in diagnosis and/or management, but a tailored shared care model is advocated.

  • Patients with ILD who are current smokers should receive opportunistic smoking cessation advice from healthcare professionals and this advice should be recorded in the clinical notes. Current smokers should be offered specialist support and nicotine replacement therapy or bupropion on NHS prescription.

  • Patients with ILD should have access to a local pulmonary rehabilitation programme.

Study Design And Population

Evaluation and Diagnosis A: Usual Interstitial Pneumonia – Interstitial Lung Disease

This study was approved by the institutional review board of Tosei General Hospital . Informed consent was not required as the data were collected retrospectively and anonymously analysed.

A retrospective review of consecutive patients with ILD evaluated at Tosei General Hospital from January 2008 to March 2013 was performed. Patients included were those who underwent an initial workup for a suspected diagnosis of IIP and whose CT scans at initial workup were available. Patients excluded were those with an indeterminate for UIP or an alternative diagnosis pattern on CT , a diagnosis of connective tissue disease , chronic hypersensitivity pneumonitis or other identifiable causes of ILD, those with concurrently associated malignant diseases, those lost to follow up within 1year of the initial workup for reasons other than death and those who did not undergo a pulmonary function test at the initial workup. All patients with clinically suspected IIP prior to SLB were included in the analysis, regardless of final diagnosis.

All patients with a UIP pattern or a probable UIP pattern on CT were included in the statistical analyses and we compared survival time and time to first AE between the two CT patterns. The same analyses were also performed in patients with a diagnosis of IPF and between IPF and non-IPF diagnoses in patients with a probable UIP pattern.

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General Management Of Idiopathic Interstitial Pneumonia

Any patients who smoke must be encouraged to stop. Supportive therapy, including access to palliative care and supplemental oxygen therapy, is important to optimise quality of life. Recent studies in interstitial lung disease have shown short-term improvements in dyspnoea, exercise capacity and quality of life following pulmonary rehabilitation,9 although this benefit appears to dissipate at six months.

Recommendations For The Management Of Sarcoidosis

  • Because of the high rate of spontaneous remission, treatment is not indicated for asymptomatic stage I disease.

  • Because of high rates of remission, treatment is not indicated in asymptomatic stage II or III disease with mildly abnormal lung function and stable disease.

  • Oral corticosteroids are the first line of therapy in patients with progressive disease determined by radiology or on lung function, significant symptoms or extrapulmonary disease requiring treatment.

  • Treatment with prednisolone 0.5 mg/kg/day for 4 weeks, then reduced to a maintenance dose which will control symptoms and disease progression, should be used for a period of 624 months.

  • Bisphosphonates should be used to minimise steroid-induced osteoporosis.

  • Inhaled corticosteroids, either as initial treatment or maintenance therapy, are not of significant benefit. Inhaled corticosteroids may be considered for symptom control in a subgroup of patients.

  • Other immunosuppressive or anti-inflammatory treatments only have a limited role in sarcoidosis, but should be considered in patients when corticosteroids are not controlling the disease or side effects are intolerable. At present, methotrexate is the treatment of choice.

  • Lung transplantation should be considered in end stage pulmonary sarcoidosis.

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Symptoms Of Interstitial Lung Disease

When you have interstitial lung disease, you cant get enough oxygen into your blood. As a result, you feel short of breath, especially when you exercise or climb stairs. Eventually, you may find it hard to breathe, even at rest.

A dry cough is another symptom. Symptoms often get worse over time.

See your doctor if you have trouble breathing. After a diagnosis, you can start treatments to manage the inflammation and scarring.

Interobserver Agreement: Ct Criteria For Uip Patterns

(PDF) Fibrosing Interstitial Lung Disease: A Practical HRCT Based ...

Walsh et al.1313 Walsh SL, Calandriello L, Sverzellati N, Wells AU, Hansell DM UIP Observer Consort. Interobserver agreement for the ATS/ERS/JRS/ALAT criteria for a UIP pattern on CT. Thorax. 2016 71:45-51. evaluated interobserver agreement for the current criteria for a UIP pattern on CT. Interobserver agreement was found to be only moderate for experienced general radiologists and thoracic radiologists, the difficulty in distinguishing among UIP patterns being attributed to discrepancies regarding the presence and distribution of honeycombing.

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