38 year old African American female with no medical problems presented to the ER with abdominal pain. This ultimately was determined to be functional (IBS vs. gastroenteritis) and has since resolved. In her workup, a CT scan was performed. With the cuts through the lungs, there was noted enlarged hilar adenopathy. The abdomen was normal.
Her PCP completed the workup by getting a CT of the chest. This revealed enlarged hilar adenopathy and she was referred to me.
She has very minimal pulmonary symptoms with the exception of dyspnea with severe amounts of exertion (i.e. running up 2 flights of stairs with groceries). She can walk really an unlimited distance on level ground. She has no cough, fevers, chills, skin rashes, ocular symptoms (an eye exam is pending), chest pain, nausea, vomitting.
SH: Works as an accountant. No exposures. No smoking
PFT's
FEV1: 2.30 (71%)
FVC: 2.78 (70%)
FEV1/FVC: 83 (100%)
TLC: 4.46 (83%)
RV: 1.50 (91%)
DLCO: 16.24 (60%)
Lab data
ACE 117 (normal 8-52)
Alk phos 147
AST 55
ALT 44
Rest of chemistries are normal
WBC 3.4
rest of CBC is normal
RADIOGRAPHS:
CT of chest with contrast:
Bilateral hilar and mediastinal adenopathy as described above with similar areas of adenopathy in the upper abdomen. This is likely on the basis of lymphoproliferative disorder although noncavitating granulomatous disease is a possibility. CT abdomen and pelvis to identify the extent of the adenopathy is recommended. Patchy areas of consolidation in both lungs particularly in the lower lobes noted. There is one nodular opacity in the right upper lobe that could also be inflammatory but it should be followed to rule out neoplastic lesion.
HRCT chest: 1. Stable hilar and mediastinal adenopathy
2. Moderate to severe areas of scarring noted in the lower lobes and to a lesser extent in the upper lobes. .
3. No areas of alveolitis or active air-space disease. No pericardial or pleural effusion seen.
For the purists:CXR: enlarged hilar adenopathy. No evidence of interstitial markings whatsoever.
She does not have a pathologic diagnosis at this time.
How would you proceed? After everyone renders their opinion, I will tell you how I proceeded.
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Ok-first, as I'm assuming that the contrast-enhanced CT of the chest and the HRCT were done within a short time frame, the "stable" hilar LAD doesn't mean much.
It all started sounding like sarcoid-mild restriction, hilar LAD, otherwise asymptomatic, elevated ACE, but then the presence of lower lobe "fibrotic" areas and the abdominal LAD may be red flags for something else. Sarcoid can cause enlarged abdominal lymph nodes, but would not be typical.
On the other hand, if someone had diffuse chest and abdominal LAD associated to a malignancy, particularly a lymphoma, or an infectious disease for that matter, I would expect some symptoms.
So, I suppose this is one of those cases of sarcoid where I'd fall on the conservative side and get tissue. In this case, I'd want surgical or core from an abdominal node first, as this could always be incidental sarcoid in a patient with lymphoma. Still, in the end, sarcoid is number one on my list.
I agree that sarcoid is so far up the list that I might get TBBx's to confirm it and then follow.
I have recently seen a case of sarcoid involving the mesenteric nodes to such an extent that the patient required surgery for an SBO. In my opinion, biopsy of the abdominal nodes would be uneccessary of the TBBX confirms the sarcoid diagnosis.
Once diagnosed, I would just follow symptoms and sprio/DLCO and if stable, would not treat with steroids.
For me, the fibrosis on HRCT does not remove sarcoid from the top 10 things on the list. Sarcoid causes fibrosis oftentimes, as ya'll know, and it's presence might be the explanation for the low DLCO.
I missed the statement that her abdominal pain had resolved. Given her current lack of symptoms, I agree that watchful waiting is appropriate. If her abdominal symptoms return or worsen, than I would re-image to see if there were any nodes that were new or increasing in size. If so, I would still obtain a biopsy. Sarcoid can cause bowel obstruction due to mesenteric LAD, but it's not the norm, and I would really hate to miss a treatable lympoma. That said, she's feeling well wihtout treatment and, as stated in my first post, the lack of associated findings makes my suspicion of lympoma pretty low.
I would also try and get tissue. Sarcoid would be up there on my list. Mesenteric TB can present with large retroperitoneal adenopathy, abdominal pain and even small ascites. Fever is not always present. Lymphoma would also be a concern.
Don't forget to rule out Lofgren's! It presents very similarly to Sarcoid. Although, the high ACE levels would suggest Sarcoid, I would want to also check the serum Ca++ level to furthur indicate sarcoid. I would assume Sarcoid but, don't forget about Lofgrens!
Did anyone ever test the Radon Gas levels in her home. i presented with the same symptoms and have now mitigated our home and am doing much better. simple. basic. Deadly Radon Gas.
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