How can we increase research in sarcoidosis?
By promoting patient and doctor sarcoidosis networks. A good example are the European Reference Networks, specifically ERN-Lung. A sarcoidosis core network has now formed within ERN-Lung with the aim of attracting more sarcoidosis research and investors.
Are people affected differently around the world?
Yes. Some examples of this are down to racial difference. People of African American, Sub-Saharan African and North African descent have many more skin lesions. People of Japanese descent tend to have more cardiac disease and those of Chinese descent more hypercalcaemia.
This is not just a racial issue as there are also regional differences. For example the Mississippi river splits the USA in half. The eastern half, the northeast and southeast have more sarcoidosis cases (about twice as much as the western half). More research is needed to understand these differences.
Is there increased risk with auto-immune diseases?
Sarcoidosis is formed by granulomas and granulomas are not seen in auto-immune diseases. People with sarcoidosis often have family members with other conditions such as lupus, rheumatoid arthritis or psoriasis. This suggests that the inflammatory genes are shared and there is evidence to support this in genetic studies.
What are sarcoidosis triggers?
The cause of sarcoidosis is unknown but research is being carried out to try and find out. It is likely that there will be a combination of genes associated with sarcoidosis. The evidence suggests that at least 3 major factors can bring on a sarcoid-like reaction:
- P-acne (a bacteria that causes acne).
- An unusual form of tuberculosis.
- Inhalation of a large amount of dust.
All these factors can lead to granulomas forming, and in the relevant genetic setting, this may be what leads to sarcoidosis. It is important to stress that sarcoidosis is not just a simple infection.
Can the sun be a trigger for sarcoidosis?
No. However, as people with sarcoidosis are at risk of hypercalcemia, sun exposure and calcium-rich foods should be limited.
Is there research about pregnancy and sarcoidosis?
There are no specific studies in sarcoidosis during pregnancy. However, we do know that certain drugs should be avoided during pregnancy, such as methotrexate. Prednisone is often given during pregnancy if there is a specific need to control an underlying disease, such as asthma. There is no evidence that any medication is needed for prophylaxis.
Is there research into cannabis therapy for sarcoidosis?
Medical cannabis is becoming more popular as a treatment for people with chronic pain syndromes, including fibromyalgia and sarcoidosis. However there are no specific studies available to show any benefits for people with sarcoidosis.
Is there research into the benefits of Frankincense?
Frankincense (olibanum) is an aromatic resin used in incense. It has anti-inflammatory properties. There is increasing evidence for the use of Frankincense in people with multiple sclerosis. However, there is currently insufficient data about its benefits for people with sarcoidosis.
Are there recommended clinics for sarcoidosis?
Yes, there are centres across the world listed on the WASOG (World Association for Sarcoidosis and Other Granulomatous Disorders.) website.
- Sarcoidosis Clinics: centres that have self-registered and show that they are familiar with sarcoidosis.
- Sarcoidosis Centres of Excellence: centres that have been through a formal, vetting process.
Not every sarcoidosis centre has gone through the process of becoming a centre of excellence. There are many doctors who take excellent care of people with sarcoidosis and have never registered. Your doctor may be well experienced in sarcoidosis, but if you are looking for a second opinion, then contact a centre of excellence.
Will I receive better care in a sarcoidosis specialist centre?
Yes. The quality of care is higher due to the greater number of patients seen and the experience gained with rare organ symptoms. Specialist centres also offer participation in clinical trials.
Is there a specialist centre for sarcoidosis in children?
Yes, but there are only a few centres worldwide. Professor Nadia Nathan at Trousseau Hospital, Paris, has experience of sarcoidosis in children.
What drugs can be used for first line therapy?
Prednisone is highlighted in the guideline as being the drug used for first line therapy.
A current research study in The Netherlands is comparing prednisone against methotrexate. It may show that methotrexate can be helpful as a first line therapy for people whose quality of life is moderately, but not severely, impaired.
How should stubborn sarcoidosis be treated?
Stubborn sarcoidosis is when someone does not improve on current therapy. To treat this, firstly find out if there is no improvement because the individual’s imaging is abnormal or because the individual still has symptoms.
If they still have symptoms and their quality of life is poor, they may want more aggressive treatment (third line therapy).
If they feel well, have few symptoms - even if the MRI scan is still abnormal, or creatine kinase (CK) is still mildly elevated – they may decide not to have more treatment. Focusing on quality of life should be the main focus with the final decision about treatment made by the patient.
How long should I be on third line therapy?
For people with chronic sarcoidosis (needing treatment beyond 2 years), for about 1 in 10 people, per year, the sarcoidosis will go away. It makes no difference if you are on methotrexate, prednisone, infliximab or third line therapy. Some people with sarcoidosis have been on drugs such as infliximab for 10-15 years.
What is the best way to treat fatigue?
Fatigue can significantly affect quality of life and it is common in sarcoidosis, being seen in at least 1 in 2 people. For some, it is an overwhelming problem. There are two recommendations in the guideline:
- To undertake a regular exercise programme three times per week.
- To consider the use of neuro-stimulants (drugs like methylphenidate and modafinil) which can help with short-term fatigue.
What is the treatment for high CRP (C-reactive protein)?
Only 1 in 5 people with sarcoidosis will have elevated CRPs (C-reactive proteins).
Most people with Lofgren syndrome have high CRP without need for use of corticosteroids. In other contexts, people whose CRP is very high, are much more likely to respond to infliximab than those whose CRP is low (although some response may also be present for this group).
These tests are only really helpful if inflammation is shown and a negative test should not stop treatment.
How long does it take for sarcoidosis to disappear?
For 1 in 3 people affected, sarcoidosis will go away. For 2 in every 3 people, sarcoidosis can be chronic and about half of those will end up on long-term treatment.
How often do I need an X-ray check-up of my lungs?
It depends on your condition and whether you are on treatment. For people without treatment and without symptoms, an annual X-ray is sufficient. For people on long-term treatment, more X-ray follow-up may be needed. Regular pulmonary function tests are also recommended. If your symptoms get worse or if complications are suspected, a chest CT should be considered.
How do you screen for multi-system organ involvement?
There is usually a healthcare checklist that your physician will use to ask about things like palpitations, headaches, vision problems, your level of breathing and shortness of breath. In addition, calcium and kidney function and liver function tests, all need to be checked regularly and at least once a year. Also, the target organs need to be monitored on a regular basis.
What is known about cardiac sarcoidosis and sudden death?
Sudden death with cardiac sarcoidosis is associated with arrhythmias (irregular heartbeat).
If you have palpitations and have had passing-out spells, you should be checked for cardiac arrhythmias due to sarcoidosis. The test is an ECG (electrocardiogram) where the heart rhythm is monitored, usually for 24-48 hours. People with known cardiac sarcoidosis arrhythmias usually have thousands of skipped beats in a day.
The ERS Sarcoidosis Treatment guideline (see Library) states that people who have arrhythmias from cardiac sarcoidosis should have an ICD (implantable cardioverter defibrillator). Anyone with known cardiac sarcoidosis should be screened for arrhythmias on a regular basis if you do not have an ICD.
Can long-term use of methotrexate have side effects?
Methotrexate is generally well-tolerated, and some people may need to take it for years. Long-term methotrexate use can increase the risk of liver toxicity which is why regular liver function tests are advised. Methotrexate should not be used during pregnancy and should be replaced with another drug for people wishing to be pregnant.
How high is the risk of steroid-induced diabetes?
Diabetes can be a complication of steroids. The higher the dose, the more problems you may have. Sarcoidosis does not directly cause diabetes, but it can cause problems with your sugar in two ways:
- Weight gain.
- Blocking the effectiveness of your body’s insulin.
If you start having problems with your sugars, your physician needs to treat it but at the same time, to think about steroid-sparing drugs.
How can I deal with weight gain and prevent diabetes?
The general recommendation is to increase and maintain physical activity i.e. burning calories and reducing fat, salt and sugar intake. Consultation with a nutritionist is advised.
What are the issues relating to Vitamin D?
Vitamin D is a complicated problem in sarcoidosis as too much Vitamin D can cause kidney stones or kidney failure.
Around 1 in 10 people with sarcoidosis have hypercalcemia which can also cause kidney stones or kidney failure. The reason for this is too much Vitamin D of the active form, called the 125 or Calcitriol. There is an enzyme in the granuloma that over-converts this and this level should be checked. Often the vitamin D25 level that is normally checked is low because it is being over-produced. Before you start vitamin D supplementation, get both the 25 OH vitamin D and D1-25 OH vitamin D checked.
In a study of over 300 patients, only 1 patient was low on their vitamin D125.
Can sarcoidosis cause gastrointestinal problems?
Gastric sarcoidosis is rare so other causes of gastrointestinal problems should be ruled out first. Symptoms include abdominal pain/discomfort, nausea, vomiting, swallowing difficulties (dysphagia), and indigestion (dyspepsia).
A simple way of avoiding gastritis when taking several drugs is to reduce the intake of food associated with gastritis and stomach pain. If gastric symptoms persist, gastroscopy is recommended. There are medications like proton pump inhibitors (omeprazole and pantoprazole) that can also be taken to prevent gastritis.
Is there a drug that has fewer liver implications?
Steroid treatment has in general few consequences for the liver. However, azathioprine, methotrexate and mycophenolate mofetil may cause inflammation in the liver. This is usually monitored by routine blood tests to check liver function.
Is adrenal insufficiency a risk if on prednisone long-term?
This relates to the amount of prednisone received. The higher the dose, the longer the secondary adrenal insufficiency (when the adrenal glands do not make enough of the hormone cortisol) is. Adrenal insufficiency is not permanent, it just means that the individual has to come off steroids slower.
Can sarcoidosis cause seasonal cough?
In sarcoidosis, cough, when present, is generally not seasonal; however, some people may have associated allergic asthma, which may flare up seasonally. Upper respiratory tract infection is a common cause of cough in winter, but people with sarcoidosis do not seem to be at higher risk for this.
What is the treatment for leg cramps?
There are two main reasons for leg cramps:
- The use of corticoid steroids.
- People with chronic respiratory symptoms hyperventilate and get leg cramps because of this.
In some cases quinine can help or a drug such as gabapentin. Speak to your primary doctor about this and have your potassium level checked.
What is the treatment for kidney stones and sarcoidosis?
Kidney stones may or may not be related to your sarcoidosis. The main reason may be due to high 125 or Calcitriol (too much vitamin D of the active form) and this should be treated as a symptom. Doctors use drugs such as hydroxychloroquine and prednisone as treatment.
My sarcoidosis is in remission but I still have problems?
General symptoms of sarcoidosis, especially fatigue, can last for many years after sarcoidosis has gone into remission in the main affected organ.
Treatment of these remaining symptoms is challenging, and anxiety and depression are common.
A second opinion from a specialist centre is strongly recommended. It may be helpful to fill in a questionnaire about fatigue (such as the Fatigue Assessment Scale), although none are specific for sarcoidosis. If other causes of fatigue are reasonably excluded, fatigue is probably due to sarcoidosis.
Can breathlessness be improved without steroid increase?
If shortness of breath continues while taking prednisone, a different medication such as methotrexate should be tried. This works about half the time but if it fails to work, a third line drug such as infliximab can be used.
Is pain in the feet a sign of sarcoidosis?
Pain in the feet could be from direct bone/joint involvement. The management of joint and bone sarcoidosis can be challenging. Methotrexate can be beneficial, although it takes several weeks to work. Physiotherapy may be recommended. If there is no response, we recommend asking the opinion of a neurologist with expertise in sarcoidosis to exclude peripheral neuropathy, which can also show with numbness, and pins and needles in the arms and legs.
Can nutrition help with pain and tiredness?
You should eat a balanced diet with plenty of fruit and vegetables. This helps to not gain weight, which can make tiredness worse. Calcium-rich foods should be limited due to the risk of hypercalcemia.
Can I have inactive sarcoidosis and continuous joint pain?
People with sarcoidosis may become inactive (or stationary) when there is no further evidence of ongoing inflammation. As a result, anti-inflammatory drugs such as prednisone and/or methotrexate are stopped.
However, some symptoms such as joint pain and fatigue can persist. This can be challenging to manage. While any joints can be affected, the feet, ankles and knees are more commonly involved. Individuals may benefit from physiotherapy.
My quality of life has fallen and my doctor has retired?
Contact another sarcoidosis specialist and continue with your regular checks, particularly because of the cardiac involvement. Corticosteroids are unlikely to improve your quality of life and sometimes they make it worse. If your sarcoidosis is under control, rather than drug treatment, your quality of life may benefit from rehabilitation. As noted in the guidelines, there are other medications to consider when prednisone and methotrexate no longer control symptoms.
Generally, the ERS Treatment Guideline is well-known among specialists. Non-specialists may not be so familiar, which is why people with sarcoidosis should be referred to specialised centres. Find out more about the ERS Sarcoidosis Treatment Guideline on our Library page.
The guideline does not cover treatment of some sarcoidosis involvements when they have not been specifically investigated by dedicated studies. For example, renal (kidney) involvement, which can be a dangerous symptom of sarcoidosis. Sarcoidosis drugs can be used in these cases but individuals should be carefully followed at a specialist centre.
The guideline provides precise treatment explanations and a clear overview of available drugs with dosage and side effects. This will help to reduce uncertainties and mistakes in the management of people with sarcoidosis.
Discuss this with your doctor and find out why they do not want to use the guideline recommendations.
Some pulmonologists may not be used to using drugs such as infliximab. In that case, you may want to ask to see a rheumatologist or someone who is used to prescribing those types of drugs for other conditions e.g., rheumatoid arthritis.
Yes, healthcare providers are not obliged to follow the guideline recommendations. However, the recommendations for use of drugs such as infliximab, usually do help healthcare providers, especially in the United States of America and other countries.
You can point out that these recommendations are produced using GRADE (The Grading of Recommendations Assessment, Development and Evaluation) and healthcare providers usually follow GRADE recommendations for conditions such as cancer.
Generally, pulmonary rehabilitation is not limited to the lung as it includes general motor activities as well. The benefit of pulmonary rehabilitation extends outside the lung, as it reduces symptoms, particularly fatigue and improves quality of life. Walking, swimming, yoga, and other low-intensity exercises may also improve quality of life for people with sarcoidosis.
Currently there is not enough research data available on these symptoms to produce a guideline. A case-by-case approach is needed and a consultation with a rheumatologist is often recommended.
This may be considered for the next guideline round if more cases and data become available.