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Interstitial Lung Disease

COVID-19: 

Sarcoidosis

Patients with sarcoidosis should already have been contacted directly and advised that they may have to shield. However many will not be in the highest risk category and do not require shielding. 

We would recommend that the following patients should be shielded:

Patients that have documented ongoing parenchymal lung disease (often referred to as stage 2,3 or 4 sarcoidosis) andare on oxygen and/orcorticosteroids (any dose) and/orimmunosuppression (including methotrexate)

We would recommend the following sarcoidosis patients do NOT require shielding:

  • Patients with a historical diagnosis of sarcoidosis but are not an treatment and are not being followed up in secondary care
  • Patients that have no documented parenchymal lung disease (often referred to as stage 0 or 1 sarcoidosis) and are not on corticosteroids equivalent to Prednisolone 20mg per day for 4 weeks or more OR corticosteroid equivalent of Prednisolone >=5mg/day for 4 weeks or more AND on other immunosuppressive therapy

For sarcoidosis patients that do not fit into these categories, the appropriate specialty consultant should be contacted for advice.

Interstitial Lung Disease

Patients with ILD should already have been contacted and advised to shield. However some will not be in the highest risk category and do not require shielding. 

We would recommend that the following ILD patients should be shielded:

Patients with have a diagnosis of IPF/probable IPF/possible IPF/working diagnosis of IPFPatients that have any form of ILD that are in one or more of the following groupsDeemed palliativeOn long term oxygenOn antifibrotics (pirfenidone OR nintedanib)On prednisolone >5mg/dayOn immunosuppressants (azathioprine, MMF, methotrexate, or on cyclophosphamide in the last 6 months)

We would recommend that the following ILD patients do NOT require shielding:

  • Patients with a historical diagnosis of ILD that are no longer on treatment for ILD and are not being followed up in secondary care

For ILD patients that do not fit into these categories, the appropriate consultant should be contacted for advice.

​Services

  • All referrals should be made to Dr Hirani, Royal Infirmary of Edinburgh or Dr Gareth Stewart, WGH
  • Use SCI-Gateway
  • Not usually urgent unless short (<3 months) onset of symptoms. Includes sarcoidosis, rare diseases such as pulmonary vasculitis and suspected occupational diseases, namely asbestosis and pneumoconiosis (occupational disease may also be referred to Dr Peter Reid, WGH)
  • CXR is essential before referral.  
  • Diagnostic terminology includes:
    • idiopathic pulmonary fibrosis (IPF)
    • hypersensitivity pneumonitis (e.g. bird fanciers lung) and
    • interstitial pneumonia
    • sarcoidosis
  • These conditions tend to be complex and can be difficult to diagnose
  • Idiopathic pulmonary fibrosis (IPF) is the commonest ILD and has an average survival from first symptoms of only 3-4yrs.  However the disease can run an unpredictable course and most patients with suspected IPF will be followed up indefinitely in the ILD clinic
  • The ILD clinic is the only local access to a specialist ILD nurse, newly licensed speciality drugs and relevant clinical trials.

Who to refer:

  • Patients of any age with suspected ILD including sarcoidosis
    • IPF typically presents in those >50 yrs with breathlessness, finger clubbing (sometimes), cough, fine bibasal velcro chest crackles and a diffusely abnormal CXR
    • Sarcoidosis typically presents in young patients (20-50yrs) with erythema nodosum, fatigue, lymphadenopathy and abnormal CXR

Who not to refer:

  • Patients who have a clear-cut cause for breathlessness eg COPD or heart failure
  • Patients with asymptomatic asbestos-related pleural plaques and otherwise normal CXR. 
  • There is guidance for managing pleural plaques under “Occupational Respiratory Disease”

How to refer:

  • All referrals should be made to Dr Hirani, Royal Infirmary of Edinburgh
  • Use SCI gateway
  • A CXR should always be performed prior to referral – please indicate where the CXR was performed. A normal CXR virtually excludes ILD
  • Spirometry is not essential prior to referral ( when performed Spirometry tends to shows a restrictive pattern)
  • All ILD referrals are triaged by a consultant or specialist ILD nurse
  • Most suspected ILD patients will be sent a health questionnaire specific to ILD prior to clinic appointment
  • The majority of suspected ILD patients will require a high resolution CT scan and detailed lung function tests that will be arranged prior to clinic visit. (will be requested by secondary care).