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Sarcoidosis

Confusing symptoms??

06/18/2007

Question:

I am a 28 year old previously fairly healthy White male. In January 2007 I developed pericarditis that advanced to tamponade, requiring a pericardial window with drainage. The pericardial fluid was described as chyleous and bloody, but was negative in all regards (negative for granuloma, neoplasm, fungus, bacteria, or viruses), and the tissue itself just showed “chronic” changes. I had a CT scan at the time (in the ED) that showed mild left hilar and multiple mediastinal lymphadenopathy, a nodule in the left middle lung, and some streaking in the left lung base. During my hospitalization for the pericardial drainage, I had moderate fevers, shortness of breath (Sp02 88% on RA), and an elevated ESR and white count.

I felt somewhat and slowly better over the course of the month following, and a repeat CT scan about 5 weeks out showed no hilar or axillary lymphadenopathy, but did still show mediastinal lymphadenopathy, right paratracheal adenopathy, and a left lingular node enlargement (largest was 1.6 x 1.8 cm I think). About 6 weeks out from surgery, my constitutional symptoms returned and have been fairly steady since then. Five months out, I still have low grade fevers (i.e., hot flashes) that last a few hours, some low level shortness of breath, and occasional pain across my anterior chest and sometimes between my shoulders posteriorly. I also have erythema across my arms and hands which sometimes looks less red and more purplish in nature. A battery of blood work (protein electrophoresis, ANA, ESR, CRP, ACE, CBC, LFT, CMP) has all been normal. My serum histoplasma CF yeast titer is elevated at 1:64. My cardiac echo is normal, and my EKG is normal. My cardiologist has pretty much dismissed me as my heart seems no longer a problem (though I do still have some pericardial-like pain).

My father has sarcoidosis but is essentially asymptomatic. My pulmonologist was concerned at one point with sarcoidosis with me, but the elevated histoplasma titers confuse things. I have been taking itraconazole 200 mg twice daily for three weeks, with no significant improvement. I did have a bizarre illness about 6 years ago with mostly peripheral neurological findings (slowed nerve conduction velocities, paresthesias, fatigue, etc.) which a neurologist diagnosed as a mild mostly sensory Gullain-Barre syndrome. In thinking back, many of those symptoms could have been attributed to sarcoid.

I am wondering if all these things could be related to sarcoid? I haven’t been able to find a case where sarcoid caused pericarditis “peripherally” (i.e., while not actually in the heart) simply by proximity to enlarged inflamed lymph nodes (like which can occur with histoplasmosis). Also, is mediastinal sarcoidosis without hilar enlargement usual? Could this all be histoplasmosis that may take longer than 3 weeks to resolve? I am confused. My docs are not at a University, but at a large medical center.

Answer:

Sarcoidosis is a diagnosis of exclusion and it is very difficult at times to differentiate histoplasmosis (infections) from sarcoidosis, which is presumably non-infectious. In fact, we have reported one case in the medical literature (see link to abstract below) wherein a patient clearly progressed from histoplasmosis to what can only be described as sarcoidosis after a long course of appropriate treatment (itraconazole).

Thus, I believe it is possible that infections can progress to sarcoidosis. How this happens is a subject of research and is not known. I would encourage you to continue treatment for histoplasmosis, but you may require alternate therapy if there is no sign of active infection (based on repeat biopsies of affected tissues, urine histoplasmosis antigens, etc.) and if granulomas typical of sarcoidosis are observed.

Related Resources:

Transformation of pulmonary histoplasmosis to sarcoidosis: a case report.

For more information:

Go to the Sarcoidosis health topic, where you can:

Response by:

Elliott D Crouser, MD Elliott D Crouser, MD
Associate Professor of Pulmonary, Allergy, Critical Care & Sleep Medicine
College of Medicine
The Ohio State University