Alexander Schult, MD

The overall death rate from Sarcoidosis is less than 5 percent. Causes of death in sarcoidosis include: progressive pulmonary fibrosis and cor pulmonale, pulmonary hemorrhage from aspergillomas developing in damaged lung tissue and myocardial involvement as a rare cause of death.

Funny, however, it is not: To me the most memorable patient with sarcoidosis was a gentleman who was a recurrent patient at the Hotel Dieu hospital in New Orleans, where I did my fellowship. His usual presentation was shortness of breath, hemoptysis and chest pain. His sole function appeared to be the education of residents and fellows about the stage IV manifestations of sarcoidosis. During a bronchoscopy, I was reminded about going spelunking (if I had ever done that)—huge cavernous areas opened up dripping mucus from what I could imagine to be stalactites, and bronchial veins engorged and oozing, trickling blood made this an unforgettable experience. Dramatic as it sounds, it also had a significant degree of impact on his life. Return hospitalizations I'm sure did not contribute to his serenity, and at one point he did require bronchial artery embolization to abate his hemoptysis. He was at one end of the spectrum.

Having worked at Martha Jefferson Hospital the last seven years and having seen a number of sarcoidosis patients, there is another end of the spectrum that is rather benign and thankfully easy on patients. With minimal side effects from medications, including methotrexate, another patient over a period of two years was able to improve what was diagnosed as nodular sarcoid (comprising approximately 5 percent of all sarcoid cases), and there was no progression of disease in terms of interstitial lung disease, worsening shortness of breath or chest pain. His bronchospasm improved, and he's now only on p.r.n. MDIs. Of note, approximately 50 percent of patients with sarcoid have bronchospasm, which may have been misdiagnosed as asthma or bronchospasm related to a different etiology prior to diagnosis of sarcoid.

The prognosis estimation can only be improved by staging and evaluation of the multisystem aspect of the disease. It has been estimated that remission occurs in approximately 60 to 80 percent of patients with radiographic stage I disease, 50 to 60 percent with stage II disease and less than 30 percent in stage III disease. It is imperative to assess the multiple systems affected in order to provide comprehensive therapy. It has also become clear to me that many patients are not interested in steroids, and that I have to offer alternatives, some of which may include doxycycline and methotrexate. continued ...

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