{"id":4055,"date":"2022-05-03T11:47:20","date_gmt":"2022-05-03T10:47:20","guid":{"rendered":"https:\/\/apps.nhslothian.scot\/refhelp\/guidelines\/interstitiallungdisease\/"},"modified":"2026-08-06T11:58:38","modified_gmt":"2026-08-06T10:58:38","slug":"interstitiallungdisease","status":"publish","type":"page","link":"https:\/\/apps.nhslothian.scot\/refhelp\/guidelines\/respiratory\/interstitiallungdisease\/","title":{"rendered":"Interstitial Lung Disease"},"content":{"rendered":"\n<h4 class=\"wp-block-heading\">Information<\/h4>\n\n\n\n<p class=\"wp-block-paragraph\">Interstitial&nbsp;lung&nbsp;disease&nbsp;(ILD)&nbsp;refers&nbsp;to&nbsp;a&nbsp;group&nbsp;of&nbsp;conditions&nbsp;that&nbsp;cause&nbsp;inflammation&nbsp;and&nbsp;scarring&nbsp;of&nbsp;the&nbsp;lung&nbsp;interstitium. This&nbsp;leads&nbsp;to&nbsp;breathlessness, cough, reduced lung function and impaired oxygen exchange.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\"><strong>Diagnostic terminology includes:<\/strong><\/p>\n\n\n\n<ul class=\"wp-block-list\">\n<li>idiopathic pulmonary fibrosis (IPF)<\/li>\n\n\n\n<li>hypersensitivity pneumonitis (e.g. bird fanciers lung)<\/li>\n\n\n\n<li>connective tissue disease associated ILD (CTD-ILD) and rheumatoid arthritis associated ILD (RA-ILD)<\/li>\n\n\n\n<li>idiopathic interstitial pneumonia (IIP)<\/li>\n\n\n\n<li>sarcoidosis<\/li>\n\n\n\n<li>rare diseases such as pulmonary vasculitis, cystic lung disease and suspected occupational diseases, namely asbestosis and pneumoconiosis<\/li>\n<\/ul>\n\n\n\n<p class=\"wp-block-paragraph\">The&nbsp;causes&nbsp;of&nbsp;ILD&nbsp;can&nbsp;vary&nbsp;widely&nbsp;and&nbsp;may&nbsp;include:<\/p>\n\n\n\n<ul class=\"wp-block-list\">\n<li>Environmental factors: Long-term exposure to harmful substances such as asbestos, silica dust, or other pollutants<\/li>\n\n\n\n<li><a href=\"https:\/\/www.mayoclinic.org\/diseases-conditions\/interstitial-lung-disease\/symptoms-causes\/syc-20353108\" target=\"_blank\" rel=\"noreferrer noopener\">Autoimmune\u00a0diseases:\u00a0Conditions\u00a0like\u00a0rheumatoid\u00a0arthritis\u00a0or\u00a0systemic\u00a0lupus\u00a0erythematosus\u00a0can\u00a0lead\u00a0to\u00a0ILD.\u00a0<\/a><\/li>\n\n\n\n<li><a href=\"https:\/\/my.clevelandclinic.org\/health\/diseases\/17809-interstitial-lung-disease\" target=\"_blank\" rel=\"noreferrer noopener\">Medications:\u00a0Certain\u00a0drugs,\u00a0including\u00a0chemotherapy\u00a0agents\u00a0and\u00a0some\u00a0antibiotics,\u00a0can<\/a> cause lung damage<\/li>\n\n\n\n<li><a href=\"https:\/\/www.mayoclinic.org\/diseases-conditions\/interstitial-lung-disease\/symptoms-causes\/syc-20353108\" target=\"_blank\" rel=\"noreferrer noopener\">Idiopathic:\u00a0In\u00a0many\u00a0cases,\u00a0the\u00a0exact\u00a0cause\u00a0of\u00a0ILD\u00a0remains\u00a0unknown.<\/a><\/li>\n<\/ul>\n\n\n\n<p class=\"wp-block-paragraph\"><\/p>\n\n\n\n<p class=\"wp-block-paragraph\"><strong>Symptoms<\/strong><\/p>\n\n\n\n<ul class=\"wp-block-list\">\n<li>Common\u00a0symptoms\u00a0of\u00a0ILD\u00a0include:\n<ul class=\"wp-block-list\">\n<li><strong>Shortness\u00a0of\u00a0Breath<\/strong>:\u00a0Especially\u00a0on\u00a0exertion,\u00a0which\u00a0may\u00a0worsen\u00a0over\u00a0time.<\/li>\n\n\n\n<li><strong>Dry\u00a0Cough<\/strong>:\u00a0A\u00a0persistent\u00a0cough\u00a0that\u00a0does\u00a0not\u00a0produce\u00a0mucus.<\/li>\n\n\n\n<li><a href=\"https:\/\/www.mayoclinic.org\/diseases-conditions\/interstitial-lung-disease\/symptoms-causes\/syc-20353108\" data-type=\"link\" data-id=\"https:\/\/www.mayoclinic.org\/diseases-conditions\/interstitial-lung-disease\/symptoms-causes\/syc-20353108\" target=\"_blank\" rel=\"noreferrer noopener\">Fatigue:\u00a0General\u00a0tiredness\u00a0and\u00a0decreased\u00a0exercise\u00a0tolerance.\u00a0<\/a><\/li>\n<\/ul>\n<\/li>\n<\/ul>\n\n\n\n<ul class=\"wp-block-list\">\n<li>Clinical signs include finger clubbing and velcro-like inspiratory crackles on chest auscultation.<\/li>\n<\/ul>\n\n\n\n<p class=\"wp-block-paragraph\"><strong>Diagnosis\u00a0of\u00a0ILD\u00a0typically\u00a0involves:<\/strong><\/p>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Medical\u00a0History\u00a0and\u00a0Physical\u00a0Examination<\/strong>:\u00a0Assessing\u00a0symptoms,\u00a0exposure\u00a0history,\u00a0and\u00a0family\u00a0history.<\/li>\n\n\n\n<li><strong>Imaging\u00a0Tests<\/strong>:\u00a0Chest\u00a0X-rays\u00a0and\u00a0HRCT\u00a0scans\u00a0to\u00a0visualise\u00a0lung\u00a0changes.<\/li>\n\n\n\n<li><strong>Pulmonary\u00a0Function\u00a0Tests<\/strong>:\u00a0To\u00a0measure\u00a0lung\u00a0capacity\u00a0and\u00a0function.<\/li>\n\n\n\n<li><a href=\"https:\/\/geekymedics.com\/interstitial-lung-disease\/\" data-type=\"link\" data-id=\"https:\/\/geekymedics.com\/interstitial-lung-disease\/\" target=\"_blank\" rel=\"noreferrer noopener\">Lung\u00a0Biopsy:\u00a0In\u00a0some\u00a0cases,\u00a0a\u00a0biopsy\u00a0may\u00a0be\u00a0necessary\u00a0to\u00a0determine\u00a0the\u00a0specific\u00a0type\u00a0of\u00a0ILD.\u00a0<\/a><\/li>\n<\/ul>\n\n\n\n<p class=\"wp-block-paragraph\"><\/p>\n\n\n\n<p class=\"wp-block-paragraph\"><strong>Treatment<\/strong><\/p>\n\n\n\n<ul class=\"wp-block-list\">\n<li>There\u00a0is\u00a0no\u00a0cure\u00a0for\u00a0most\u00a0forms\u00a0of\u00a0ILD,\u00a0treatment\u00a0focuses\u00a0on\u00a0managing\u00a0symptoms\u00a0and\u00a0slowing\u00a0disease\u00a0progression.\u00a0Options\u00a0may\u00a0include:<\/li>\n\n\n\n<li><strong>Medications<\/strong>:\u00a0Corticosteroids\u00a0and\u00a0other\u00a0immunosuppressive\u00a0drugs\u00a0to\u00a0reduce\u00a0inflammation, anti-fibrotics to target lung scarring and slow disease progression.<\/li>\n\n\n\n<li><strong>Oxygen\u00a0Therapy<\/strong>:\u00a0To\u00a0help\u00a0maintain\u00a0adequate\u00a0oxygen\u00a0levels\u00a0in\u00a0the\u00a0blood.<\/li>\n\n\n\n<li><strong>Pulmonary\u00a0Rehabilitation<\/strong>:\u00a0A\u00a0program\u00a0that\u00a0includes\u00a0exercise\u00a0training\u00a0and\u00a0education\u00a0to\u00a0improve\u00a0lung\u00a0function\u00a0and\u00a0quality\u00a0of\u00a0life.<\/li>\n\n\n\n<li><a href=\"https:\/\/www.mayoclinic.org\/diseases-conditions\/interstitial-lung-disease\/symptoms-causes\/syc-20353108\" data-type=\"link\" data-id=\"https:\/\/www.mayoclinic.org\/diseases-conditions\/interstitial-lung-disease\/symptoms-causes\/syc-20353108\" target=\"_blank\" rel=\"noreferrer noopener\">Lung\u00a0Transplant:\u00a0In\u00a0severe\u00a0cases,\u00a0a\u00a0lung\u00a0transplant\u00a0may\u00a0be\u00a0considered.\u00a0<\/a><\/li>\n<\/ul>\n\n\n\n<p class=\"wp-block-paragraph\"><\/p>\n\n\n\n<p class=\"wp-block-paragraph\"><strong>Prognosis<\/strong><\/p>\n\n\n\n<ul class=\"wp-block-list\">\n<li>The\u00a0prognosis\u00a0for\u00a0individuals\u00a0with\u00a0ILD\u00a0varies\u00a0widely\u00a0depending\u00a0on\u00a0the\u00a0specific\u00a0type\u00a0of\u00a0disease,\u00a0its\u00a0cause,\u00a0and\u00a0how\u00a0early\u00a0it\u00a0is\u00a0diagnosed.\u00a0<\/li>\n\n\n\n<li>These conditions tend to be complex and can be difficult to diagnose.<\/li>\n\n\n\n<li>Idiopathic pulmonary fibrosis (IPF) is the commonest ILD and has an average survival from first symptoms of only 3-5yrs.\u00a0 However\u00a0the disease can run an unpredictable course and most patients with suspected IPF will be followed up indefinitely in the ILD clinic.<\/li>\n\n\n\n<li>The ILD clinic is the only local access to a specialist ILD nurse, newly licensed speciality anti-fibrotic drugs and relevant clinical trials, therefore early referral to the ILD service is essential.<\/li>\n<\/ul>\n\n\n\n<p class=\"wp-block-paragraph\"><strong>L.N &amp; D.M &#8211; 6-8-26<\/strong><\/p>\n\n\n\n<div class=\"wp-block-getwid-tabs\" data-active-tab=\"0\"><ul class=\"wp-block-getwid-tabs__nav-links\"><\/ul>\n<div class=\"wp-block-getwid-tabs__nav-link\"><span class=\"wp-block-getwid-tabs__title-wrapper\"><a href=\"#\"><span class=\"wp-block-getwid-tabs__title\">Referral Guidelines<\/span><\/a><\/span><\/div><div class=\"wp-block-getwid-tabs__tab-content-wrapper\"><div class=\"wp-block-getwid-tabs__tab-content\">\n<h4 class=\"wp-block-heading\">Who can refer<\/h4>\n\n\n\n<p class=\"wp-block-paragraph\">Any primary or secondary care clinician can refer.<\/p>\n\n\n\n<h4 class=\"wp-block-heading\">Who to refer:<\/h4>\n\n\n\n<ul class=\"wp-block-list\">\n<li>Refer via sci-gateway\n<ul class=\"wp-block-list\">\n<li>Patients of any age with suspected ILD including sarcoidosis\n<ul class=\"wp-block-list\">\n<li>IPF typically presents in those &gt;50 yrs with breathlessness, finger clubbing (sometimes), cough, fine bibasal velcro chest crackles and a diffusely abnormal CXR<\/li>\n\n\n\n<li>Sarcoidosis typically presents in young patients (20-50yrs) with erythema nodosum, fatigue, lymphadenopathy and abnormal CXR<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n\n\n\n<h4 class=\"wp-block-heading\">Who not to refer:<\/h4>\n\n\n\n<ul class=\"wp-block-list\">\n<li>Patients who have a clear-cut cause for breathlessness eg COPD or heart failure<\/li>\n\n\n\n<li>Patients with asymptomatic asbestos-related pleural plaques and otherwise normal CXR. See\u00a0<a href=\"https:\/\/apps.nhslothian.scot\/refhelp\/guidelines\/respiratory\/asbestosrelateddiseases\/\" target=\"_blank\" rel=\"noreferrer noopener\">Asbestos Related Diseases \u2013 RefHelp<\/a><\/li>\n<\/ul>\n\n\n\n<h4 class=\"wp-block-heading\">How to refer:<\/h4>\n\n\n\n<ul class=\"wp-block-list\">\n<li>All referrals for suspected ILD should be made using Sci-Gateway \u2013 RIE or WGH or SJH &gt; Respiratory medicine &gt; LI Basic Sign Referral and freetext reason for referral in presenting complaint.<\/li>\n\n\n\n<li>A CXR should always be performed prior to referral. A normal CXR virtually excludes ILD.<\/li>\n\n\n\n<li>Spirometry is not useful without gas transfer for ILD so please don&#8217;t book spirometry in suspected ILD. The ILD clinic will book full PFT when triaging the referral.<\/li>\n\n\n\n<li>The vast majority of ILD is not urgent. The only exceptions are of rapidly progressing ILD (worsening symptoms within a few months) or suspected pulmonary vasculitis.<\/li>\n\n\n\n<li>All ILD referrals are triaged by a consultant or specialist ILD nurse.<\/li>\n<\/ul>\n\n\n\n<h4 class=\"wp-block-heading\">After referral:<\/h4>\n\n\n\n<ul class=\"wp-block-list\">\n<li>Most suspected ILD patients will be sent a health questionnaire specific to ILD prior to clinic appointment, please let patients know to expect this.<\/li>\n\n\n\n<li>The triaging Respiratory clinician will often organise a high resolution CT scan and detailed lung function tests prior to clinic review, so patients can be informed they may receive letters for these appointments prior to clinic.<\/li>\n<\/ul>\n<\/div><\/div>\n\n\n\n<div class=\"wp-block-getwid-tabs__nav-link\"><span class=\"wp-block-getwid-tabs__title-wrapper\"><a href=\"#\"><span class=\"wp-block-getwid-tabs__title\">Primary Care Management<\/span><\/a><\/span><\/div><div class=\"wp-block-getwid-tabs__tab-content-wrapper\"><div class=\"wp-block-getwid-tabs__tab-content\">\n<ul class=\"wp-block-list\">\n<li>Early diagnosis is paramount in the management of ILD. Please have a low threshold for requesting a CXR in patients with SOBOE and a persistent dry cough.<\/li>\n\n\n\n<li>Please consider referring ILD patients with SOBOE to pulmonary rehabilitation.<\/li>\n\n\n\n<li>Please consider referring patients with advanced ILD to community palliative care for symptom control.<\/li>\n\n\n\n<li>Please signpostpatients to the Action for Pulmonary Fibrosis website for further information, <a href=\"https:\/\/actionpf.org\" target=\"_blank\" rel=\"noreferrer noopener\">https:\/\/actionpf.org<\/a><\/li>\n<\/ul>\n<\/div><\/div>\n\n\n\n<div class=\"wp-block-getwid-tabs__nav-link\"><span class=\"wp-block-getwid-tabs__title-wrapper\"><a href=\"#\"><span class=\"wp-block-getwid-tabs__title\">Resources and Links<\/span><\/a><\/span><\/div><div class=\"wp-block-getwid-tabs__tab-content-wrapper\"><div class=\"wp-block-getwid-tabs__tab-content\">\n<ul class=\"wp-block-list\">\n<li>Action for Pulmonary Fibrosis Website (<a href=\"https:\/\/actionpf.org\" target=\"_blank\" rel=\"noreferrer noopener\">https:\/\/actionpf.org<\/a>)<\/li>\n\n\n\n<li><a href=\"https:\/\/actionpf.org\/information-and-support\/what-is-pulmonary-fibrosis\" target=\"_blank\" rel=\"noreferrer noopener\">What is pulmonary fibrosis<\/a><\/li>\n\n\n\n<li><a href=\"http:\/\/apps.nhslothian.scot.nhs.uk\/refhelp\/guidelines\/ResourcesLinks\/Respiratory%20Clydeside%20Action%20on%20Asbestos%20%28clydesideactiononasbestos.org.uk%29.aspx\" target=\"_blank\" rel=\"noreferrer noopener\">Action on Asbestos<\/a><\/li>\n\n\n\n<li><a href=\"http:\/\/www.clydesideactiononasbestos.org.uk\/\" target=\"_blank\" rel=\"noreferrer noopener\">www.clydesideactiononasbestos.org.uk\/<\/a><\/li>\n\n\n\n<li><a href=\"https:\/\/actionpf.org\/information-and-support\/what-is-asbestosis\" target=\"_blank\" rel=\"noreferrer noopener\">https:\/\/actionpf.org\/information-and-support\/what-is-asbestosis<\/a><\/li>\n\n\n\n<li><a href=\"https:\/\/www.nice.org.uk\/guidance\/conditions-and-diseases\/respiratory-conditions\/pulmonary-fibrosis\" target=\"_blank\" rel=\"noreferrer noopener\">Pulmonary fibrosis | Topic | NICE<\/a><\/li>\n\n\n\n<li><a href=\"https:\/\/www.asthmaandlung.org.uk\/healthcare-professionals\/ild\/diagnosing-ild\" target=\"_blank\" rel=\"noreferrer noopener\">Diagnosing patients with Interstitial Lung Disease (ILD) | Asthma + Lung UK<\/a><\/li>\n\n\n\n<li><a href=\"https:\/\/www.thoracic.org\/statements\/insterstitial-lung-disease.php\" target=\"_blank\" rel=\"noreferrer noopener\">Interstitial Lung Disease<\/a><\/li>\n\n\n\n<li><a href=\"https:\/\/services.nhslothian.scot\/respiratory\/respiratory-managed-clinical-network-mcn\/\" data-type=\"link\" data-id=\"https:\/\/services.nhslothian.scot\/respiratory\/respiratory-managed-clinical-network-mcn\/\" target=\"_blank\" rel=\"noreferrer noopener\">NHS Lothian Respiratory Managed Clinic Network (MCN) Guidance<\/a><\/li>\n<\/ul>\n<\/div><\/div>\n<\/div>\n","protected":false},"excerpt":{"rendered":"<p>Information Interstitial&nbsp;lung&nbsp;disease&nbsp;(ILD)&nbsp;refers&nbsp;to&nbsp;a&nbsp;group&nbsp;of&nbsp;conditions&nbsp;that&nbsp;cause&nbsp;inflammation&nbsp;and&nbsp;scarring&nbsp;of&nbsp;the&nbsp;lung&nbsp;interstitium. This&nbsp;leads&nbsp;to&nbsp;breathlessness, cough, reduced lung function and impaired oxygen exchange. Diagnostic terminology includes: The&nbsp;causes&nbsp;of&nbsp;ILD&nbsp;can&nbsp;vary&nbsp;widely&nbsp;and&nbsp;may&nbsp;include: Symptoms Diagnosis&nbsp;of&nbsp;ILD&nbsp;typically&nbsp;involves: Treatment Prognosis L.N &amp; D.M &ndash; 6-8-26<\/p>\n","protected":false},"author":2,"featured_media":0,"parent":4381,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","meta":{"_searchwp_excluded":"","footnotes":""},"categories":[597],"class_list":["post-4055","page","type-page","status-publish","hentry","category-interstitiallungdisease"],"publishpress_future_workflow_manual_trigger":{"enabledWorkflows":[]},"rttpg_featured_image_url":null,"rttpg_author":{"display_name":"NHS Lothian","author_link":"https:\/\/apps.nhslothian.scot\/refhelp\/author\/nhs-lothian\/"},"rttpg_comment":0,"rttpg_category":" <a href=\"https:\/\/apps.nhslothian.scot\/refhelp\/category\/respiratory\/interstitiallungdisease\/\" rel=\"tag\">Interstitial Lung Disease<\/a>","rttpg_excerpt":"Information Interstitial&nbsp;lung&nbsp;disease&nbsp;(ILD)&nbsp;refers&nbsp;to&nbsp;a&nbsp;group&nbsp;of&nbsp;conditions&nbsp;that&nbsp;cause&nbsp;inflammation&nbsp;and&nbsp;scarring&nbsp;of&nbsp;the&nbsp;lung&nbsp;interstitium. This&nbsp;leads&nbsp;to&nbsp;breathlessness, cough, reduced lung function and impaired oxygen exchange. Diagnostic terminology includes: The&nbsp;causes&nbsp;of&nbsp;ILD&nbsp;can&nbsp;vary&nbsp;widely&nbsp;and&nbsp;may&nbsp;include: Symptoms Diagnosis&nbsp;of&nbsp;ILD&nbsp;typically&nbsp;involves: Treatment Prognosis L.N &amp; D.M &ndash; 6-8-26","_links":{"self":[{"href":"https:\/\/apps.nhslothian.scot\/refhelp\/wp-json\/wp\/v2\/pages\/4055","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/apps.nhslothian.scot\/refhelp\/wp-json\/wp\/v2\/pages"}],"about":[{"href":"https:\/\/apps.nhslothian.scot\/refhelp\/wp-json\/wp\/v2\/types\/page"}],"author":[{"embeddable":true,"href":"https:\/\/apps.nhslothian.scot\/refhelp\/wp-json\/wp\/v2\/users\/2"}],"replies":[{"embeddable":true,"href":"https:\/\/apps.nhslothian.scot\/refhelp\/wp-json\/wp\/v2\/comments?post=4055"}],"version-history":[{"count":8,"href":"https:\/\/apps.nhslothian.scot\/refhelp\/wp-json\/wp\/v2\/pages\/4055\/revisions"}],"predecessor-version":[{"id":29405,"href":"https:\/\/apps.nhslothian.scot\/refhelp\/wp-json\/wp\/v2\/pages\/4055\/revisions\/29405"}],"up":[{"embeddable":true,"href":"https:\/\/apps.nhslothian.scot\/refhelp\/wp-json\/wp\/v2\/pages\/4381"}],"wp:attachment":[{"href":"https:\/\/apps.nhslothian.scot\/refhelp\/wp-json\/wp\/v2\/media?parent=4055"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/apps.nhslothian.scot\/refhelp\/wp-json\/wp\/v2\/categories?post=4055"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}