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– Don’t be a slave to imaging when evaluating the patient with sarcoidosis.

“Sometimes, the worst-looking patients [on imaging] have the best prognosis,” Daniel Culver, DO, said at the annual Congress of Clinical Rheumatology. Patients with Löfgren’s syndrome are a very good example of this tenet, he said in an interview. Scans can look alarming, with multiple widespread granulomas. But Löfgren’s is generally a benign condition, despite its threatening mien.

The video associated with this article is no longer available on this site. Please view all of our videos on the MDedge YouTube channel


Instead of imaging, “Let two things drive your decision to treat: danger to an organ, and quality of life,” said Dr. Culver, a pulmonologist and director of the Sarcoidosis Center of Excellence at the Cleveland Clinic in Ohio; he is also president of the World Association for Sarcoidosis.

He agrees with a decision schema published in 2015 (Clin Chest Med. 2015;36[4]:751-67).

Six factors weigh in favor of treatment:

  • Symptomatic disease.
  • Impaired organ function.
  • Disease endangering an organ.
  • Progressive disease.
  • Clear-cut disease activity.
  • Low likelihood of remission.

These must be balanced – with patient input as the fulcrum – against five factors that favor conservative management:

  • Minimal symptoms.
  • Good organ function.
  • Low risk of danger to organs.
  • Inactive disease.
  • Higher likelihood of remission.

The decision to embark on a treatment program, usually starting with a steroid-based regimen, can’t be taken lightly, Dr. Culver said. A 2017 study showed that steroids pose a cumulative risk of toxicities for sarcoidosis patients (Respir Med. 2017 Nov;132:9-14). Patients who started steroids faced more than a doubling in the risk of a toxic side effect by 96 months when compared with those who didn’t. But even short-term steroid use increased the risk of a toxicity, Dr. Culver said. The study noted that problems can begin to occur in as little as 1 month, at a cumulative dose as low as 1 g.

 

 


For patients who fall onto the “treat” side of the risk teeter-totter, Dr. Culver recommended starting with an initial course of prednisone at 20-30 mg daily for no more than 4 weeks. Responders can taper to less than 10 mg/day. Those who continue to do well can maintain low-dose prednisone for up to 12 months and then complete the taper. Patients who relapse can add an immune modulator (methotrexate, azathioprine, leflunomide, or mycophenolate).

Those who have an inadequate response to the initial prednisone course should then get an immune modulator. If they do well, that can be maintained; a second modulator can be brought on board if necessary.

For those who don’t respond at all to the initial prednisone course, it’s necessary to proceed immediately to an immunosuppressive regimen to prevent irreversible fibrosis.

Dr. Culver noted associations with multiple pharmaceutical companies, but said none were relevant to his talk.

SOURCE: Culver D. CCR 2018.

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– Don’t be a slave to imaging when evaluating the patient with sarcoidosis.

“Sometimes, the worst-looking patients [on imaging] have the best prognosis,” Daniel Culver, DO, said at the annual Congress of Clinical Rheumatology. Patients with Löfgren’s syndrome are a very good example of this tenet, he said in an interview. Scans can look alarming, with multiple widespread granulomas. But Löfgren’s is generally a benign condition, despite its threatening mien.

The video associated with this article is no longer available on this site. Please view all of our videos on the MDedge YouTube channel


Instead of imaging, “Let two things drive your decision to treat: danger to an organ, and quality of life,” said Dr. Culver, a pulmonologist and director of the Sarcoidosis Center of Excellence at the Cleveland Clinic in Ohio; he is also president of the World Association for Sarcoidosis.

He agrees with a decision schema published in 2015 (Clin Chest Med. 2015;36[4]:751-67).

Six factors weigh in favor of treatment:

  • Symptomatic disease.
  • Impaired organ function.
  • Disease endangering an organ.
  • Progressive disease.
  • Clear-cut disease activity.
  • Low likelihood of remission.

These must be balanced – with patient input as the fulcrum – against five factors that favor conservative management:

  • Minimal symptoms.
  • Good organ function.
  • Low risk of danger to organs.
  • Inactive disease.
  • Higher likelihood of remission.

The decision to embark on a treatment program, usually starting with a steroid-based regimen, can’t be taken lightly, Dr. Culver said. A 2017 study showed that steroids pose a cumulative risk of toxicities for sarcoidosis patients (Respir Med. 2017 Nov;132:9-14). Patients who started steroids faced more than a doubling in the risk of a toxic side effect by 96 months when compared with those who didn’t. But even short-term steroid use increased the risk of a toxicity, Dr. Culver said. The study noted that problems can begin to occur in as little as 1 month, at a cumulative dose as low as 1 g.

 

 


For patients who fall onto the “treat” side of the risk teeter-totter, Dr. Culver recommended starting with an initial course of prednisone at 20-30 mg daily for no more than 4 weeks. Responders can taper to less than 10 mg/day. Those who continue to do well can maintain low-dose prednisone for up to 12 months and then complete the taper. Patients who relapse can add an immune modulator (methotrexate, azathioprine, leflunomide, or mycophenolate).

Those who have an inadequate response to the initial prednisone course should then get an immune modulator. If they do well, that can be maintained; a second modulator can be brought on board if necessary.

For those who don’t respond at all to the initial prednisone course, it’s necessary to proceed immediately to an immunosuppressive regimen to prevent irreversible fibrosis.

Dr. Culver noted associations with multiple pharmaceutical companies, but said none were relevant to his talk.

SOURCE: Culver D. CCR 2018.

 

– Don’t be a slave to imaging when evaluating the patient with sarcoidosis.

“Sometimes, the worst-looking patients [on imaging] have the best prognosis,” Daniel Culver, DO, said at the annual Congress of Clinical Rheumatology. Patients with Löfgren’s syndrome are a very good example of this tenet, he said in an interview. Scans can look alarming, with multiple widespread granulomas. But Löfgren’s is generally a benign condition, despite its threatening mien.

The video associated with this article is no longer available on this site. Please view all of our videos on the MDedge YouTube channel


Instead of imaging, “Let two things drive your decision to treat: danger to an organ, and quality of life,” said Dr. Culver, a pulmonologist and director of the Sarcoidosis Center of Excellence at the Cleveland Clinic in Ohio; he is also president of the World Association for Sarcoidosis.

He agrees with a decision schema published in 2015 (Clin Chest Med. 2015;36[4]:751-67).

Six factors weigh in favor of treatment:

  • Symptomatic disease.
  • Impaired organ function.
  • Disease endangering an organ.
  • Progressive disease.
  • Clear-cut disease activity.
  • Low likelihood of remission.

These must be balanced – with patient input as the fulcrum – against five factors that favor conservative management:

  • Minimal symptoms.
  • Good organ function.
  • Low risk of danger to organs.
  • Inactive disease.
  • Higher likelihood of remission.

The decision to embark on a treatment program, usually starting with a steroid-based regimen, can’t be taken lightly, Dr. Culver said. A 2017 study showed that steroids pose a cumulative risk of toxicities for sarcoidosis patients (Respir Med. 2017 Nov;132:9-14). Patients who started steroids faced more than a doubling in the risk of a toxic side effect by 96 months when compared with those who didn’t. But even short-term steroid use increased the risk of a toxicity, Dr. Culver said. The study noted that problems can begin to occur in as little as 1 month, at a cumulative dose as low as 1 g.

 

 


For patients who fall onto the “treat” side of the risk teeter-totter, Dr. Culver recommended starting with an initial course of prednisone at 20-30 mg daily for no more than 4 weeks. Responders can taper to less than 10 mg/day. Those who continue to do well can maintain low-dose prednisone for up to 12 months and then complete the taper. Patients who relapse can add an immune modulator (methotrexate, azathioprine, leflunomide, or mycophenolate).

Those who have an inadequate response to the initial prednisone course should then get an immune modulator. If they do well, that can be maintained; a second modulator can be brought on board if necessary.

For those who don’t respond at all to the initial prednisone course, it’s necessary to proceed immediately to an immunosuppressive regimen to prevent irreversible fibrosis.

Dr. Culver noted associations with multiple pharmaceutical companies, but said none were relevant to his talk.

SOURCE: Culver D. CCR 2018.

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