Providing Support to Sarcoidosis and Lyme Disease Communities. A group of ladies got together in August 2011 to discuss their journey and experiences with Sarcoidosis and one who possibly has Lyme Disease. As a result Sue Sherratt decided to develop a web site for Sarcoidosis and Lyme Disease Support in Australia. This blog is a result of that meeting. You can reach Sarcoidosis Australia by emailing firstname.lastname@example.org.
Sarcoidosis is a chronic, multi-system disease that has the ability to affect any organ in the body, but mostly the lungs and lymph glands. The disease is a growing issue in the U.S., as 2.4% of African Americans and 1% of all Caucasians will develop the disease during their lifetime. Sarcoidosis is not a discriminatory disease and affects both men and women of all ages and half of all Americans living with the disease experience no noticeable symptoms before diagnosis. It is therefore vital that all patients become better educated on the disease. Daniel Culver DO, a Staff Physician in the Department of Pulmonary, Allergy, and Critical Care Medicine at Cleveland Clinic, provides answers to questions concerning the diagnosis and treatment of sarcoidosis.
Dr. Culver’s clinical interests include sarcoidosis, pulmonary alveolar proteinosis, critical care, and interstitial lung diseases
Cleveland_Clinic_Host:Welcome to our Online Health Chat with Dr. Daniel Culver. We are thrilled to have him here today for this chat. Thank you for joining us Dr. Culver, let’s begin with the questions.
Speaker_-_Daniel_Culver: Thank you for having me. We received a lot of questions and I will try my best to get through all of them.
Sarcoidosis: What is it?
lakshami:Dear Dr., I am newly diagnosed with sarcoidosis in the lungs and exocrine glands (salivary and lacrimal glands) with very minimal symptoms. I only have mild aches in my entire body. While looking for information on the Internet about sarcoidosis, I have stumbled upon various forums where sarcoidosis patients regard spontaneous remission as a myth or a misleading term. Is it true? Do you know any case where spontaneous remission was achieved (without any drugs)? They also say that it never goes away for good that it always comes back with more severity. Is it true? I have read many medical websites that talk about spontaneous remission with regard to stages, but these are experiences of real people. Should I be worried? Please let me know. I was told by healthcare professionals that I don't need any treatment and it will hopefully go away within two-three years on its own, and I was happy to hear this. But after reading these forums, my world has turned upside down and I am losing hope. I am only 29. Thank you.
Speaker_-_Daniel_Culver: Spontaneous remission rarely does occur. The chances of it, overall, are at least 50% and may be higher in certain areas of the world, such as Scandinavia. Even if there is not remission, the disease may not cause problems, just being evident on x-ray or testing. I would not say that it comes back after remission, almost ever. Also, currently we do not know of any medicines that achieve spontaneous remission. We typically use medicines to either control bothersome symptoms or when progressive disease is threatening an important organ, but not when trying to induce remission.
ritumehraa:Hello. I have been diagnosed with sarcoidosis - stage 2. I had a gallium scan too, which showed Panda sign after 24 hrs and partial Lamda after 48 hours. The rest of the scan (including lungs) was unremarkable. My x-ray showed stage 1, but the CT scan showed a few pulmonary infiltrates as well (which made the diagnosis as stage 2). I also have a few skin lesions (look like pimples, 3-4 on face). A biopsy was taken from a lesion and showed a lone granuloma. After abdominal ultrasound, I also have mild splenomegaly (13.2 cm) and slightly coarse echogenicity in liver parenchyma. I am confused as I have been told that gallium shows inflammation where ever it is in the body. Why didn't it show up in my lungs and skin? Secondly, what are the chances of spontaneous remission (if such thing exists as real life people with sarcoid regard it as a false term) in stage 2. I don't have any symptoms except very slight shortness of breath after exertion and mild unilateral nasal congestion. Thanks.
Speaker_-_Daniel_Culver: Please see the answer to lakshami's question regarding remission.
We can predict the likelihood of remission by some features of the sarcoidosis. The most commonly used predictive indicator is the Scadding scale, which is based on a chest x-ray, not on s chest CT. If you have organ involvement outside the lungs, such as a enlarged spleen, then the chance of remission is slightly lower. In terms of the Gallium scan, it is not very sensitive. PET scanning is probably a better test - but also not 100% sensitive in showing unsuspected organ involvement. However, we do not typically get PET scans because they rarely change our management and are expensive, except in the case of cardiac sarcoidosis.
clevelandborn:Most people start with lung problems being noted. How does one monitor the other organs to know the disorder has not spread?
Speaker_-_Daniel_Culver: Monitoring sarcoidosis is mostly still a low tech operation. We know that if we look hard, we can find sarcoidosis in many more organs nowadays than in the past. A good example of this is the PET scan. But the practical issue is; what would we do with the information, even if you knew an organ was involved? Since most asymptomatic organ involvement never causes problems, we take the stance of not looking with a fine-toothed comb for lesions that are not causing problems. The exception to this is that eye involvement should be screened for, at least at the time of diagnosis. How aggressively to look for asymptomatic heart involvement is very controversial.
cdjohns1:Is it that critical to know your "stage"? I note that some patients seem really hung up on their stage. But in my reading, the stage seems to matter little…
Speaker_-_Daniel_Culver: Scadding stage (a method of describing lung involvement in sarcoidosis) refers to a chest x-ray pattern that gives a very loose approximation of the chances for a spontaneous remission, within 5 years after the diagnosis. I agree that people focus too much on 'their stage.' It is important to remember that many patients with stage 1 disease may have a poor prognosis, and vice-versa. Also, one does not proceed through the stages sequentially over time and the stages are not validated for CT.
otoolejim:My doctor is having me take CT Scans every 3-6 months to monitor whether my lymph nodes have changed in shape or level of inflammation. Is this common? I am confused, what is sarcoid and should I be worried about it being fatal?
Speaker_-_Daniel_Culver: This gets to the issue of how to monitor pulmonary sarcoidosis. I generally try not to react to changes in the lymph node size that are not causing problems. So if the diagnosis is clear, than usually symptoms, breathing tests and plain x-rays should be enough. This will save you a lot of radiation exposure.
leg222:If I'm coming in from out of town to the Cleveland Clinic as a new patient with suspected but not confirmed Sarcoidosis, what can I expect as far as the diagnostic process? Will I be at the Clinic for several days?
Speaker_-_Daniel_Culver: For any patient coming to a tertiary center, it is important that you have a conversation beforehand with a clinician, in order to clarify the goals of the visit. Typically, both a diagnosis and a treatment plan can be set up within several days in a center like the Cleveland Clinic. Pre-scheduling based on goals of the visit will be key.
otoolejim: I have enlarged or inflamed, lymph nodes and it is thought to be Sarcoid. My question is: Does weather affect the condition? (i.e. I am contemplating a move from Chicago to Central Florida. Will the heat and humidity cause the condition to worsen?)
Speaker_-_Daniel_Culver: I don't expect weather to affect your lymph nodes. Sometimes patients with sarcoidosis experience asthma-like symptoms and these may be worse in the heat.
MartinJ:Dr. Culver, I have bad allergies and systemic sarcoidosis. Should I get allergy shots or am I playing with the immune system too much? I am weaning off prednisone and started Plaquenil® (Hydroxychloroquine sulfate) one month ago. Also, vitamin D came back very low on blood work and because I have taken prednisone for awhile, my rheumatologist is blasting me with Vitamin D and Calcium with D. I heard no D or calcium supplements for sarc patients. Please Help! I feel like I am shooting in the dark hoping to hit the mark. Thanks.
Speaker_-_Daniel_Culver: Vitamin D deficiency is common in sarcoidosis patients, but you have to be careful about replacing vitamin D and taking extra calcium. It would be important for your doctor to monitor the active form of vitamin-D (dihydroxy vitamin-D) at least at the beginning of therapy. Most patients can take it, however, without a problem.
cdjohns1:The "active form" would be the 1,25D lab result?
SarcoidLady:What is sarcoid-induced asthma and how is it different from 'regular' asthma?
Speaker_-_Daniel_Culver: Asthma like symptoms are very common in sarcoidosis, and in fact, there is a big overlap between the two. Fortunately, we can usually treat airway symptoms from sarcoidosis or asthma with similar approaches. I don't know of a way to definitively distinguish between two, sarcoid-induced asthma and 'regular' asthma.
HasHope: Is it known if treatment ever causes a worsening of sarcoid - either during treatment or after stopping treatment, please? Thank you.
Speaker_-_Daniel_Culver: This has been a theory for some time, but in my view it is unproven. There are some authors who espouse this view. For example, recent paper by Johan Grunewald's group suggested that patients with acute sarcoidosis and certain genetic backgrounds were more likely to have persistent sarcoidosis, if they were treated with steroids. However, similar to every other study on this issue, it may simply be that those patients had more severe disease and therefore were predestined for a worse course.
blessed1:I recently came across the theory that calcium, Vitamin D and the sun cause sarcoidosis to get worse. Is this true? I informed my doc yesterday and she didn’t know anything about this.
Speaker_-_Daniel_Culver: This theory is popular on some websites. The science behind it is not accepted by the mainstream sarcoidosis community. Vitamin D has both pro and anti-inflammatory effects and the rigorous research necessary to prove this hypothesis has not yet been conducted.
dianeinlargo:I have stage 4 sarc and was in the hospital in April for pneumonia. I still have not fully recovered and am wondering how long it takes to fully recover?
Speaker_-_Daniel_Culver: By this time, I would be concerned about whether or not there will be an atypical organism that does not respond to typical antibiotics or if your symptoms are due to typical active sarcoidosis. I would normally expect typical pneumonia to have resolved by now.
redding114:Can sarcoidosis be mistaken for cancer? I guess my question is, can this turn into cancer if it spreads outside the lungs?
Speaker_-_Daniel_Culver: Sometimes cancer can cause granulomas to form nearby or in regional lymph nodes. So the diagnosis of sarcoidosis depends on both a compatible biopsy and a consistent clinical picture. There are some data also that suggests patients with chronic sarcoidosis have a slightly higher chance of developing a lymph node cancer, known as lymphoma. I don't think that sarcoidosis spread outside the lungs indicates that it will turn into cancer.
otoolejim:I am a Type 2 diabetic and I understand treatment would include steroids. What would this do to my blood sugars, and are there other methods without steroids?
Speaker_-_Daniel_Culver: Steroids are probably overused in sarcoidosis. There are a wide variety of other options for patients who do not respond, or have intolerable side effects from steroids. I think many of these have a lower risk profile than commonly used steroid doses. You should find a doctor who is comfortable in using a range of options.
TRESTE:In the case of my Pulmonary Sarcoidosis, I suffer from awful chest pain (mainly left sided), which I read other patients also suffer from. I have been on opioids previously and now I am off these and on Pregabalin only, which is not covering pain sufficiently. I am told that even after transplant this pain may still exist - Is this your experience??
Speaker_-_Daniel_Culver: Chest discomfort is very common in sarcoidosis, but we don't know what causes it. In one study, it was not related to the size of the lymph nodes or any other feature of the chest imaging. Sometimes treatment of neuropathic pain with Pregabalin (Lyrica®) can be helpful and sometime immunosuppressant’s that we use for other manifestations are helpful, but not always. I am not sure about whether the pain persists after transplant. I certainly hope that after going through something like a transplant, that it would be gone.
rouse:I am a pulmonary patient in Perth, Australia. I have end stage Pulmonary Sarcoidosis. The disease has proven resistant to everything and has damaged my lungs beyond a sustainable level. I have Pulmonary Sarc/Fibrosis, some bronchiostasis, pulmonary hypertension (PH) on exercise, and also M.A.C. (Mild & untreated). I was diagnosed in 2007, initially IPAH (on exercise) was diagnosed as disease but then Sarc found on CT. I have been on every drug known to help & some unproven meds. Currently on Cellcept® (Mycophenolate) - 1.5 grams amongst others (PH not treated). Pain is also a major issue - taking Pregabalin 225mg for this. On O2 24/7. I have just undergone transplant workup for double Lungs, however it sounds as if they may have found some disease in left side of heart. If this is the case, can lung tx still be done in the hope that immune suppression will slow the disease? If not, then do you know of any other drugs that might help for both Cardiac & Pulmonary Sarcoidosis.? Somebody suggested Iloprost, but I cannot find anything on this other than for P.H. (pulmonary hypertension)?
Speaker_-_Daniel_Culver: In a patient with lung and heart involvement contemplating a lung transplant, my bias is that I would want to be confident that I can suppress the inflammation in the heart before proceeding with a lung transplant. The question of whether you would need a heart and lung combined transplant would depend on the status of the heart. We have found the cardiac PET scan to be fairly helpful in assessing the degree of inflammation in the heart and monitoring the response to therapy. If you can suppress the inflammation then you can be relatively confident that the transplant meds will do the same. If the heart involvement is very extensive now, I would suggest considering a combined heart and lung transplant. With regards to Iloprost (Ventavis®), there is one report showing modest improvements with quality of life. I would be concerned about using pulmonary vasodilators if there is significant heart disease. This should be discussed carefully with your doctor.
starkl24:How do you know for sure if you are in remission?
Speaker_-_Daniel_Culver: I think it is probably impossible to know for sure. But from a pragmatic point of view, if you have no need for treatment for several years, usually three, we can say that there is practical remission. In the strictest sense of the word, we would like to see a normal chest x-ray and no evidence of active inflammation. Many patients have an abnormal chest x-ray with inactive disease and will never have a problem with their sarcoidosis.
Harmles2:After 10 yrs with sarcoidosis, I went into remission about 6 months ago. I had been on MTX & prednisone, which completely shut down my immune system, I had many infections (staph, respiratory) which resulted in me being on more than a dozen 14-30 days periods of various antibiotics. I think the antibiotics are what actually drove the sarc into remission. What is the latest research on using antibiotics to treat sarcoidosis? Also, I'm aware that a study has shown bacteria in 90% of BAL from sarc patients. Doesn't this support antibiotic use? (PS I do not think the Marshall protocol is sound, but my own experience says antibiotics work!)
Speaker_-_Daniel_Culver: This is an important question that comes up often. We still don't know for sure what causes sarcoidosis. I suspect that there may be more than 1 agent that triggers the disease. In the case you describe with this many infections, I would want to be sure that there are no immune deficiency syndromes that can mimic sarcoidosis, including have pulmonary granulomas.
There has been a fair bit of research in recent years suggesting that microbacteria or other organisms might trigger sarcoidosis. Whether treating these will cause sarcoidosis to regress is unproven. In the past, treating with drugs for garden variety tuberculosis has not been effective. One argument against bacteria being a cause of sarcoidosis is that patients do not develop overwhelming super-infections, even when treated with very powerful immunosuppressant’s. So, the jury is still out and it is an area of active research.
Harmles2:Arava® (Leflunomide) seems to work well with less side effects, is this moving ahead of prednisone as a treatment option?
Speaker_-_Daniel_Culver: We have had very nice success with Leflunomide (Arava®). I do not think it is ahead of Prednisone, but it is a very nice alternative as a steroid sparing agent.
TRESTE:What is your knowledge of Tacrolimus for treatment of End Stage Pulmonary Sarcoidosis, given failure of all other available medication?
Speaker_-_Daniel_Culver: Tacrolimus (Prograf®) and its cousin cyclosporine should work very well in theory. They both inhibit lymphocytes. However, the clinical studies of cyclosporine have been very disappointing and that fits with my clinical experience. There is very little data about Tacrolimus (Prograf®), but the enthusiasm for it is low, based on the cyclosporine story.
cjs6a:Has there been benefits from treating sarcoidosis with Cimzia®?
Speaker_-_Daniel_Culver: Cimzia® is called certolizumab, and it is part of a class of newer drugs that block tumor necrosis factor. I am not aware of any data for certolizumab (Cimzia®)and sarcoidosis. However, two related drugs, infliximab (Remicade®) and adalimumab (Humira®) have been shown to be effective for sarcoidosis. There are certain features that predict a higher chance of response to infliximab (Remicade®), including the presence of chronic disease, severe disease, and certain extra pulmonary manifestations.
eperry16: How do you treat cardiac sarcoidosis?
Speaker_-_Daniel_Culver: This is a very difficult issue. Monitoring activity for cardiac sarcoidosis is extremely controversial for medications; steroids are usually the mainstay of treatment. There is a Japanese series that suggests 1/2 mg per kg of Prednisone is just as good as higher doses, but not all experts agree with this. It is also important to strongly consider placing a defibrillator in any patient with active cardiac sarcoidosis. When to stop therapy and how to adjust it requires very careful and close monitoring. We have used other agents as well, but we always start with Prednisone.
redding114:What is the highest dose of prednisone they can put you on? And can you take Methotrexate along with it for treatment?
Speaker_-_Daniel_Culver: Some experts advocate using doses as high as 60-80 mg of Prednisone daily for cardiac or neurologic sarcoidosis. However, in our experience most patients do not require doses this high. For the lungs, 20-40 mg daily is recommended as a starting dose by most experts. We think that most patients do not require more than 30 mg daily as a starting point for lung sarcoidosis. We have a goal, which must be individualized, of reaching a maintenance dose of 10 mg per day, or less. We commonly use other medications, such as Methotrexate, in conjunction with Prednisone often, as a means to reduce the Prednisone dose. Methotrexate may require up to 6 months to be fully effective.
Cleveland_Clinic_Host:Thank you again Dr. Culver for taking the time to answer our questions about Sarcoidosis.
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