Chapters Transcript Sarcoidosis Diagnosis: Bronchoscopic Modalities Course: Sarcoidosis: Challenges and Progress I'm gonna be talking about sarcoidosis diagnosis and specifically bronchoscopic modalities to get us the pieces of tissue we need. I also have nothing to disclose. So my objectives. All right. So really, we don't have a specific diagnostic test for sarcoidosis. As we learned in our first section, there's really not a significant finding that's going to say, OK, this is 100% sarcoidosis. So what do we do? We employ these diagnostic algorithms, which really involve Three major criteria. One, a compatible clinical presentation. So what are those? Sometimes we see Lofgren syndrome, so bilateral hyal lymphadenopathy, erytheminodosum, fever, and oftentimes migratory polyarthralgias in young women. I've never seen this, but hear about it, um, you'll see here for syndrome. So I like to use the acronym PUF parotid gland enlargement, uveitis, facial nerve palsy, and fever. lupus purnow, I'm gonna be, I guess, the first of many to show this picture today, um, and these are gonna be bluish red or violacious papules or plaques on the nose, cheeks, or ears. And again, just to mention our bilateral hyal lymphadenopathy and oftentimes asymptomatic patients, um, this would be a stage one, scatting stage one sarcoidosis. And then sometimes we still see these gallium scans and you're gonna have your lambda sign, which is the pattern of uptake of your metacinal and hilar lymph nodes, and your pandaine, your bilateral lacrimal salivary gland uptake of the gallium. Those syndromes oftentimes do not require any tissue diagnosis. That's your diagnosis, you can stop there. The second part of this algorithm is to establish non-case and granulomas, which we heard about, um, from Doctor More, and really not all granulomas are created equal. You need um a well formed non-aziaating that is most specific for sarcoid. And then excluding other granulomas diseases, including paramalignant granulomaous inflammation. And establishing systemic involvement. Oftentimes sarcoidosis does not involve one single organ system. And you can use a variety of tools, including the WaAG organ um instrument to establish your multi-system disease. OK. So to stage or not to stage by chest X-ray, we heard a little bit about Scanning, who established these um stages actually in 1970. So stage one, again, you're gonna have your hylo lymphadenopathy. Stage two, you're gonna have your adenopathy plus some sort of pulmonary infiltrated process. Stage 3, you lose your lymph nodes, and stage 4, you progress to fibrosis. Hopefully, we don't get there. But do we really need scanning stage anymore, or should we be doing high res CT? I think if I were to pull the audience, almost all of us are going to say, or our ED colleagues are going to get the CAT scan before we do, um, that our patients are getting these high res CTs. So, it's getting actually a little bit outdated. Um, that's my daughter, it's her 100th day of school today. I promised I would show that. Um, and this paper by Doctor Meyer essentially is saying that we can use our high resolution CTs as a sort of radiographic biomarker. They're gonna tell us more about prank abnormalities. They're gonna tell us more about airway abnormalities, about the surrounding vascular structures. They're also gonna tell us, is the disease reversible or irreversible? Is there fibrosis there? And we can also use them to prognosticate disease course. All right, so this slide essentially was the updated ATS um guideline for diagnosis and detection of sarcoidosis. And again, similar to what I just said, it's saying patients in whom there's a high clinical suspicion for sarcoidosis, I went through some of those syndromes, um, ATS guidelines say no sampling of lymph nodes is required. For patients with asymptomatic bilateral hylar lymphadenopathy, it's a little less clear, um, but no recommendations for or against. If you wanted to exclude other processes, perhaps going forward and sending these patients for diagnosis makes sense. And then patients who have suspected sarcoid and lymphadenopathy, um, for whom you've determined they absolutely need a tissue diagnosis. The suggested diagnostic modality of choice is endoronchial ultrasound for lymph node sampling. And this is Fevered um above media synoscopy for your initial mediasinal um and or higher lymph node sampling. So, really, Ibis kind of took sarcoid diagnosis by storm. There was the 2013 granuloma randomized clinical trial, which really showed a high diagnostic yield, um, of 87%. It's less invasive. This is comparing me cynoscopy, and then really combination with standard bronchoscopic techniques, trans bronchial biopsies and the bronchial biopsies are both safe, feasible, and improves your diagnostic yield. You actually Approach a 100% yield in EIS plus transbronchial biopsy. There's no difference with rapid onsite. yield is associated with stage, which makes sense, right? Um, one is actually greater than 2, and then also increases with two or more lymph node stations. There's no difference in your 21 versus 22 gauge needles, but you're gonna get your highest sensitivity with the larger bore of 19 gauge. Now metocynoscopy does have a higher diagnostic yield, yes, but higher complication rates extrapolating from lung cancer meoidal staging data. Um, and again, just to feature the granuloma randomized clinical trial from JAMA in 2013. So why do conventional bronchoscopy if you can just do Ibis? Well, again, I said, it increases your yield. Um, before we had EIS, we were looking at yields between 37 to 90% with poor with pure transbronchial biopsy. Obviously higher yield with higher stage because you're gonna have um more lymph parmal involvement. And higher yield with number of passes. You really should be doing 4 to 7. Endobronchial biopsy, variable yield 20 to 61%. Obviously, if you do see cobblestoning, right, or endoronchial granuloma is inflammation, you're gonna have a higher yield, 54% to 91%. Multiple sites, different, um, multiple passes, different sites. The cry as are oftentimes emphasized as your best target. And then if you're in there, why not do a BAL, right? We have a CD4 to CD8 ratio that's often greater or equal to 3.5, low sensitivity but high specificity, excuse me, um, and then you're going to have a higher yield with flow cytometry. We actually send, um, oftentimes either a BAL fluid or pooled lymph node for flow cytometry, and that will get us our CD4 to CDA ratio. And then since you're there, you can send your BAL cultures and exclude granulomas infections, such as fungal or mycobacterial infections. This is actually a retrospective study from Seoul, Korea. Again, looking at the diagnostic yield, you're going to see it's the highest with IBIS and TBNA, but when you add the transbronchial biopsy, you're getting even higher and up to 100%. If you added in a bronchial biopsy, in addition, it's also quite high. I do want to say though, when they compared Ebis alone to the addition of the modalities, it actually was not statistically significant. So Ebis really is the standard of care. OK, I'm gonna go through a few cases. Um, so, case one, we have a 50 year old male smoker. He has a history of a brain tumor, some sort of brain tumor that was resected in 2008, complicated by seizure. He's referred for evaluation of pulmonary nodularity and significant mesinal and hylar lymph nodes. So you can see here he has subcrial lymph node and a right periatracheal lymph node, which we sampled with EES, the lower pictures. And um we got non-caseating granuloma and we actually were able to get his records from, oh sorry, his CD4 to CD8 ratio was also elevated, and his records actually showed this brain tumor um in 2008 actually had multiple non-caseating granulomas. So he is diagnosed with multi-system sarcoidosis um and placed on immunosuppressive therapy. Case 2. We have a 53 year old female. Recent diagnosis of optic neuritis of unknown ideology, treated with pulse steroids. She had a CT chest obtained as part of her evaluation and showed scattered intrathoracic lymph nodes. So again, I'm showing the CT showing subcrial bilateral hilar nodes. We took her for EIS. Seely, again, not all granulomas are created equal, but we found necrotizing granulomas. Um, but we excluded infection with the optic neuritis, clinically this made sense, um, and she was treated, actually, just with a brief course of steroids to treat the optic neuritis was totally asymptomatic from the pulmonary perspective. So she's off of treatment and just being monitored for now. Her CD4 CD8 ratio was 3.6 to 1, so just above that upper limited normal range. OK, case 3. So 69 male, former smoker. We um talked a little bit about World Trade Center exposure, having an increased risk. This patient had World Trade Center exposure, found to have numerous mass-like consolidations in his right upper and lower lobes, along with hylermitacial lymphadenopathy. So we took him for TBBX transbronchial biopsy. We also did Ebis of his right hylar lymph node, and what we found was alveolar lung parrankima with patchy interstitial fibrosis. Um, and it poorly formed granuloma. So again, then we're starting to think is, could this be HP, um, and he was monitored very closely, actually wound up getting rebiopsied several months later and found to have cancer. So again, we also need to keep on our differential. Could this be paragranulomaous inflammation or sarcoidosis and potentially a cancer or here potentially HP and a cancer. Case 4 hot off the press. I did this Tuesday, um, in the OR. We have a 55-year-old male in stage renal, non-obstructive CAD had severe mitral stenosis, so his induction was a nightmare, but he got through it. Um, also a peripheral eosinophilia, and we were consulted to perform bronchoscopy because he had an abnormal chest CT, made a sino hyal lymph nodes, and they wanted this done before they replaced his valve. So we took him, we did left upper lobe and left lower lobe tissue um via transbronchial biopsy approach. You could see, see in the middle picture, he had some evidence for cobblestoning potentially, so we took some endoronchial tissue. And he had pretty significantly enlarged lymph nodes. Station 7, 11R, and 4L were sent for histopathology and flow cytometry. I was hoping to have pre-limb stuff, but by the time we got out of the OR Tuesday night, um, unfortunately we weren't able to get anything. But again, high suspicion for potential sarcoid here. Oftentimes we don't. Um, we will, I should say, oftentimes you can see peripheral eosinophilia. You often will not see it, um, in your pathology specimens, and we did send his BAL and you can see, um, he didn't have marked eosinophilia in his um bronchial fluid. All right, how can we improve our diagnostic yield? What do we do with those patients who don't have those infiltrated process, these sort of small peripheral pulmonary nodules? What do we do with them? So, Could potentially robotic bronchoscopy help us with this, and absolutely. um this is looking at a precise study um which essentially was multicenter really for lung cancer diagnosis but found 82% yield with nodules between 1 to 2 centimeters, 85% yield with nodules between 2 to 3 centimeters. This is a picture we um actually got an ion robot to our Bellevue campus, um, over a year ago now, and this is the first picture with the team in the OR, so it was exciting. So, again, we've had the I on a little over a year. I am not interventially trained, but um to do the training, so um this is just some preliminary data looking at my um success compared to some of the um Essentially compared to Super D data from the Navigate study, and you can see I've only done 22 cases so far, so this is very preliminary, um, but I am showing some success in getting diagnosis and isn't just for lung cancer. I have shown some sarcoidosis cases. So again, this is just data looking at the different areas we're diagnosing the number of lesions, the size of the lesion. Again, the average lesion size 2.35, so we can get out there and target these small nodules and particularly if malignancy is in your differential, it's really important that we get there. OK, so just have a few more minutes. Um, some of the robot cases I've done. So 41 year old male, history of neurosyphilis treated in 2021. He had chronic granulomatous panuveitis of unknown ideology with variable disease severity since 2017, would be in and out of the hospital for flares of this panuveitis, and he was found to have some pulmonary fisroba nodules. So we said, OK, let's take him, let's see if we can get this and get him a diagnosis for his panuveitis. Um, oh, right, the pathology didn't come in there, but I will just tell you. So, on his, um, biopsies of his right upper lobes, so I was able to navigate and you can actually do a simulation outside if you have time, you should have time later. Um, and I was able to diagnose a rare non-necrotizing granuloma, um, in the right upper lobe, and we also did lymph node biopsies. So we gave him a diagnosis of sarcoidosis, um, and at least a ideology for his pan uveitis and screening for, um, additional systems involved. He was actually discharged on cellsep. More recently had a flare and is being worked up for cardiac sarcoid because of increasing dyspnea on exertion. This is K 6, um, 67 year old female history of CVA and thyroid mass. The thyroid mass was biopsied non-diagnostic, unfortunately. Came in with pulmonary nodules and perifisal mass-like consolidation. So you can see here in the planning stage, um, I actually did not do, um, this right when it was so visually based, and as you can see, there's no great airway going to it. When you're planning, you can plan and see what areas go there. Sometimes you actually can extend an airway if you think it goes further. But the one on that right side really did not have, um, a great navigational pathway. Um, we did get to the left upper lobe and we're able to show acute and chronic inflammation. Um, unfortunately, we did not get any granulomas. Um, she had anthrochotic lymph nodes on her ibis. So her diagnosis is still a little bit in the air, um, and we are actually undergoing a PET scan to see if there's additional targets for her. I just wanted to go back really quick because I think I glossed over my CAT scan findings. Really quick. Yes, I glossed over this slide. I got excited with the picture of Millie, um. Again, in addition to what we can show on X-ray, it's so important that we get these disease characteristics, um, of sarcoid, right? Because I said it we're, if we want to get these peril lymphatic nodule targets, we got to get these CTs. Um, and again, just a ploy that actually sarcoid could look like anything on chest CT. I often, when I'm teaching the residents say it's the syphilis of inflammatory diseases that really can do anything. But the patterns that are common, again, are the perillymphatic nodules, the And we're seeing it from hypersensitivity pneumonitis, which is also been central lobular, bronchial wall thickening, groundla opacities, and these prankable masses that you think are are cancer. Maybe they're not, or could they be again, peronnulose inflammation, but we got to get there. We got to get tissue sometimes for these cases. Um, and then obviously fibrosis and traction bronchiectasis with our stage 4 disease, um, and oftentimes a mid to upper zone predominance. And then this galaxy sign. Um, which is the coalescence of small granulomas to create this nodular opacity resembling in the galaxy. So conclusions. In patients with a high clinical suspicion for sarcoidosis, I went over those syndromes, Lofgren's, lupus perineal, Herefordt syndrome, tissue diagnosis may be unnecessary. If you think there can be something else going on, obviously, even those patients you are going to refer for biopsy. In patients with asymptomatic bilateral hylolymphadenopathy, It's not clear whether or not you need tissue diagnosis. Um, obviously, it should be a discussion with the patient. Um, and if there was evidence for an organ involved, such as neuro or cardiac, you may wanna send those patients for diagnosis because that will, um, you know, affect treatment. And a bronchial ultrasound, um, for lymph node sampling is preferred over meta synoscopy, even though a relatively lower yield, but much less complication. And then additional bronchoscopic modalities really do improve your diagnostic yield and should still be used. These are transbronchial biopsy, particularly of stage 2 or 3 disease, and a bronchial biopsy, particularly if you have cobblestoning. And BAL for CD4 to CD8 and then really we need to be cognizant of potentially accessing these really peripheral based nodules if cancer is on a differential to exclude cancer and potentially give our patients who have multi-system disease an answer um and a clear path for treatment. So robotic assisted bronchoscopy is really the new frontier and can fac facilitate sampling of these small peripheral nodules. And then, it's a tradition after every um symposium, I take a picture with my family. So, um, these are the pictures of the prior year, and I misspoke. We're actually, it was 4 years ago, jeez, it's not 2025, it's um 2020 when we were here right before the pandemic hit. Um, but yeah, so I'll have a new picture for next year, and a reminder that our symposium will be Friday, February 28th of 2025. Thank you. Published March 7, 2024 Created by Related Presenters Kerry Hena, MD View full profile
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