Steroid responsive adenopathy?

65-year-old woman presented initially for evaluation of shortness of breath. A CT thorax revealed some patchy airspace opacification RUL (see below) . She underwent bronchoscopic evaluation and it demonstrated some interstitial inflammation with hyperplastic type 2 pneumoctyes. No granulomas seen. No fungal or AFB elements on BAL. A cell count was not sent.
She was begun on a course of steroids 40mg and felt her breathing improved significantly while on them, but once she stopped them, her shortness of breath recurred. Her cough has also recurred, though it's mostly non-productive. A CT was repeated 5 monts later and is shown below.



Question: What ILD had adenopathy and is steroid responsive? The only 2 I can think of are sarcoid and berrylliosis, but the interstial inflammation would be inconsistent with that. Any thoughts out there?

A man with weakness and a some minor CT abnormalities

From Doug:

53 yo man with an 80 pack yr smoking hx and history of Etoh abuse was admitted
with a 5 week hx of progressive ataxia, diplopia, dysarthria,
and dysphagia. Initially w/u revealed unremarkable MRI brain, and a CT
C/A/P showing prevascular lymph nodes with some calcifications and left hilar borderline enlarged lymph nodes, and a renal cyst not further characterized on that study.

DDx included subacute cerebellar degeneration and progressive supranuclear palsy.
Multiple CSF and serum labs were pending at time of discharge

He was readmitted a week later for progressive dysphagia for liquids in addition to solids, worsening gait s/p falls, and continued dysarthria and diplopia. IV IgG was given empirically without improvement. Repeat LP and additional infectious
serologies were negative. A dedicated renal CT was done to further characterize the indeterminate lesion on the previous CT, showing only a
simple cyst.

What would you do next? <<<>>>





Addendum added after case presented:
These are the EBUS images from the bronch including and the core biopsy from the TBNA. The answer was that these little lymph nodes were small cell carcinoma. The special stain is a cytokeratin stain of the malignant cells.

Shortness of breath with phrenic nerve paralysis

Submission
60 year old woman with sarcoidosis by mediastinal biopsy 1978 doing well, never requiring steroids. 2 mos prior developed SOB - smothering type of feeling even at rest but also on exertion. A CXR during a routine physical showed a left paralyzed hemidiaphragm.

Pmhx
sarcoid as above
NQWMI 1991 Echo on f/u showed torn chordae and LV "contractile abnormality" on a thallium test.


Three fluorouscopies revealed unilateral paralysis. Scheduled for EMG studies

Patient also adds:
"I am 60 y/o and live a quiet life but my legs are now getting weak and I am unable to bend down in a sitting position and get up. I failed to mention that I had a lumbar puncture which was normal. MRI showed cervical problems but nothing serious I guess. The pons area of my brain showed vascular changes due to aging. I also had strabismus during all of this and had a prism put in my glasses because of a convergence problem with my eyes. Could this all be connected? Myasthenia gravis I guess has been ruled out and they are calling this idiopathic phrenic nerve paralysis. Should I pursue this any further?I have about 40% function of my left lung."

Note: please consider adding a comment to help keep this blog going!

Shortness of breath

Submission from outside reader:

Hi I have been looking at this blog for since 2005 on a weekly basis and wonder if you take questions from patients. Your site is informative it is a pity you are not getting the hits.

I am a 38yo M and I have a chronic symptoms now for many years with NO DIAGNOSIS despite pressing for one.

I have a hacking cough with some sputum in the morning, I also have nearly constant SOB with an inability to get a deep breath. A feeling of stiffness in my lungs. I feel constantly like my breathing is not good. This has been with me since 2003. Excercise tolerance is ok i.e. I could run a mile in 10min but I struggle to breathe throughout.

Pleurisy type pain on and off weekly, specifically located in same location at upper left back. Lower rib pain on left.

Asthma as a child, allergic rhinitis as a teenager and young adult, smoked in college (10 a day) and socially (30 at weekend) till I was 33. On and off asthma meds since 1994. Tried Singulair, Symbicort, Spiriva etc. Tried also extensive courses of Zoton, Nasocort etc.

Have been evaluated many times over the years by 3 pulmos spent '000$ and the best they can give me as a diagnosis is " a chronic inflammation similar to chronic bronchitis with little or no asthma but we are reluctant to give it a title "

Here is the dilemma, all tests normal my FEV1 is 4.45…101%, FVC 5.8…104%, FEV/FVC 76. DLCO Normal. FEF25-75 83% Lung Vols Normal


Here is the question:

Why do I have symptoms if all tests are normal. What do you guys think.. have you seen this before, normal tests but chronic symptoms with dyspnea.

Chest xray x 4 Normal
2 x HRCT (1 year between) Normal, Body Ples Normal. Methacholine Normal
ECG Stress Test Normal
Echo Cardio Normal
Bloods Normal, 2 x HRCT (1 year between) Normal, Body Ples Normal. Methacoline Normal

MRI ordered by Rheumo .. Normal

Not in my brain either!! I have loads of other stuff to worry about like kids, my stressful job as a broker, mortgages etc

Pulmicort discontinued?

Has anyone else heard that Astra Zeneca will be discontinuing pulmicort? Were they not making a profit on this? Anecdotally, I see a lot of patients on Pulmicort.

This is from The FDA site:

NDA 20-441, PULMICORT TURBUHALER 200 mcg (budesonide inhalation powder) will no longer be available at AstraZeneca.

The introduction of PULMICORT FLEXHALER (budesonide inhalation powder, 90 & 180 mcg), NDA 21-949, will be accompanied by the phasing out of PULMICORT TURBUHALER so as to minimize confusion in the marketplace.

AstraZeneca has no further plans to manufacture NDA 20-441, PULMICORT TURBUHALER 200 mcg (budesonide inhalation powder).

Worsening dyspnea in a patient with IPF

71 year olf woman with idiopathic pulmonary fibrosis diagnosed by open lung 2004 (UIP on biopsy). Since that time she has been stable with good exercise tolerance, and able to get around with little difficulty. PFT's also stable during that time frame with FVC in low 80% since 2004. Over the last 2 months she reports increased dyspnea on exertion and fatigue. Her ambulation has decreased over the last 3 or 4 months because of pain of her left hip, which for the last 6 months she had been getting injections for. She is due to get left total hip replacement. She has difficulty getting out of the chair because of pain in her hip. She does still try to get around, but her ambulation she does admit has decreased over the last 3 months because of this pain. She denies any nocturnal shortness of breath or orthopnea. She has no chest pain. There is no fevers, chills, night sweats. No appreciable weight gain subjectively. No anorexia. No cough.

Her physical exam is a pleasant woman sitting in a chair, in no acute distress. Blood pressure 123/59, pulse is 77, respiration is 20, O2 sat is 97% on room air, her weight is 228 pounds. Her weight in March 21st on another scale at another clinic was 221 pounds and her weight on our scale in January 2007 was 231 pounds. HEENT: Trachea midline, no lymphadenopathy. The JVP seems to be about 4 to 5 cm. The lungs have crackles basilarly and laterally about the third of the way up. Heart is regular rate and rhythm with a positive S3, no murmurs or rubs. Abdomen: Benign. There is trace pitting edema bilaterally at the ankles.

Spirometry shows the ratio of 84%. FEV1 of 1.87 (83% of predicted), FVC of 2.21 (74% of predicted). DLCO 65% of predicted. In May 2006 FVC was 80% of predicted and DLCO 74% of predicted.
CXR this visit:


CXR from 2 years ago:


What are your thoughts and/or what would you do next?

Acute on chronic Blogemia

Sorry there have been no new posts. Baleeiro and I are essentially the last surviving members of the blog (functionally, not literally - the others are not dead; they just lost interest) and our motivation for daily posts has somewhat diminished given lack of participation from outside readers, previous contributors and anyone else who might come across this blog. I will most likely leave a post or two a week and we will see where this goes. We have been steadily building a database over 2 years of pulmonary cases (with discussion) and all categorized, so it is a bit of a shame that it goes this way. Hopefully we can wean the blog off of the proverbial vent and not proceed to comfort care measures....

-JJ

low DLCO in runner

Submission from Hal :


15 y/o WF distance runner on track team c/o SOB. No improvement in the past year as perceived inability to breathe. She is is 5 7 tall, 115 pounds. All State, All American runner in 1600M and 800M.

PFTs showed DLCO 71% pred (18.9 with ref 26.6), DL Adj 80% (18.9 with 23.5 ref).
VA 5.09 L

FVC 106%, FEV1 100%, FEF25-75 96%.

Chest xray showed...ordered but not done yet.

Normal CBC

Any thoughts on etiology and suggestions for further work-up?

Cavitary mass

86 year old man with history of dementia brought by his caretaker for mental status changes and lethargy. He lives in an assisted living facility, but has no TB risk factors otherwise (He has his own apartment-like structure, but I beleive there is a common area).

His CT:



There was no hilar or mediastinal adenopathy.
The thickest wall area on that lesion was 4-5 mm.

Someone placed a PPD and it was 11 mm. It was reportedly negative 6 months ago.
He is not productive of sputum, so 3 gastric lavages were done and they were all negative. What would you do next? Are you worried about active tb? Here are my thoughts
1. How sensitive is gastric lavage for afb?
2. location of the cavitary mass is not where tb should be.
3. is he a ppd converter, or was he anergic or are we seeing a booster effect
4. His family does not want an aggressive w/u for cancer, but on the other hand one would not want to send someone home to hospice with active tb....

Dose of T-PA - Part Deux

I didn't get much feedback or interest on the post below on T-PA and empyemas so I contacted our T-PA rep and we got some references on the matter.
The dose varies widely and it seems you just can't go wrong...
In the Chest abstract I had listed in the original post the authors used 10 mg per instillation.
In a brief review/case report in Hospital Pharmacy in 2003 (vol 38 (11); pp: 1024-29) the authors used 16mg/100ml NSS infused daily. They derived the dose by extrapolating the ratio of Streptokinase usually used in empyemas to the dose for MI's.
In another Chest abstract, the initial dose was 10mg for complicated hemothoraces and that was increased to 25mg, still with good tolerability.
The pediatric literature was a bit more consistent and usually 0.1mg/kg was used in various trials.
Any other suggestions?

Dose of T-PA

There is a wealth of articles on using fibrinolytics in empyema (with mixed results) listing the doses for urokinase and streptokinase. I have had trouble finding a narrower consensus on T-PA dosing though. The Pediatric literature suggests ~4mg/kg and there is a dose of 2mg for clotted central venous access. I also found an abstract from Chest where doses of 10 mg were used but couldn't find a good consensus.
Have you used T-PA in empyemas? And how much?

Lung mass(es)

Unfortunately, our computer is not letting me retrieve the PET images on this patient so I'll try and describe it without puppets.
This is a 71 y/o man with long TOB Hx but still good mechanics (FEV1~78%) with a new lung mass. He was found to have a 2.5-cm mass in the RML and had a CT-guided Bx which was + for adenoCa. He then had a PET and came to see us: the mass was obviously hot but he had FDG-avid subcarinal and L hilar nodes and a 1-cm FDG-avid LUL peripheral lesion. I bronch'ed him and sampled his nodes and got lots of giant cells and lymphocytes but no malignancy (from both sites). A LUL BAL was non-Dx.
Would you Bx the LUL separately? And if so how would you approach this (synchronous vs metastatic lesions, etc.)?

Tongue lesions followed by dyspnea?

From AK:

22 year old with recurrent dyspnea, usually preceded by pea-sized painless denuded areas on the tongue (see picture). Episodes of dyspnea have landed her in the ER multiple times, where she's treated with nebs and steroids. No ulcers anywhere else on her body, but she has had some easy bruising lately. Others describe wheezing on exam and I've heard inspiratory stridor during an episode. Normal VC movement on laryngoscopy. PFTs with mild flattening of inspiratory portion of flow-volume loop, otherwise unremarkable. Normal soft tissue film of the neck. Methacholine challenge normal. CXR normal. Allergy skin testing negative. IgE 5. TSH normal. CRP 0.4. Liver and kidney function normal. U/A normal. CBC with diff normal.

Also had an unexplained episode of weight loss last fall, approx. 20 lbs. Resolved spontaneously and weight came back.

I'm temped to call this VC dysfunction, but other docs claim definite peripheral wheezing on exam, and these tongue lesions don't ring any bells.

Any ideas?

Where is Waldo (or the line)?

As ICU co-directors we get to see some unusual situations. This line was reportedly a "little traumatic" going in and there is no good blood return from any of the ports. I enlarged the segment of interest and crudely followed the trajectory. Where is the line going?

Rheumatoid arthritis and effusions

From one of our readers. Edited a slight bit.

Found your site and thought I‘d run some of this by you to get your take on the situation so we might be more well-equipped to talk to our local doctors.

Husband is a 52 yr old smoker (about a pack and a half a day) with advanced rheumatoid arthritis. He is on:
Methotrexate for ten years (20 mg a week, currently)
Had a five-year stint on enbrel/etanercept - discontinued six months ago)
Recently moved to remicade.
Other meds: folic acid, indomethacin, prednisone 30 mg, QD, and hydrochlorthiazide (12.5 mg, daily).

February 2005, he started feeling “full” in his torso. Blood tests were “normal”. But he gained 9 pounds in a day. In the ER he was fpund to have fluid around his heart and in his pleural cavity. They withdrew 31 ounces of fluid off the heart. All seemed well....for a time.

Had another bout with all this in March, 2005.

Around Christmas 2005, he started feeling full again, and the doctor said go to the Emergency Room. Hubby said he didn’t want to go through all that again, so they told him to take prednisone (40 mg a day). Gave him relief. Started to step-down the dosage of prednisone...got down to 10mg/day, and started feeling full, again.

HRCT was ordered and showed:

Clinical Information: Pleural effusion.

Findings: Standard and high resolution chest CT images demonstrate moderate centrilobular and paraseptal emphysema. No focal consolidating process. Mild ground glass with traction bronchiectasis involving the peripheral aspect of the lung fields, with upper lung zone predominance. The findings are nonspecific, and may represent sequela of chronic hypersensitive pneumonotis. There is evidence of prior granulomatous disease. There is a 5-mm hyperdense pulmonary nodule in the right upper lobe; this nodule may be partially calcified. There is also an 8-mm, not obviously calcified nodule in the right middle lobe. Small left and trace right pleural effusions are noted. There is no lymphadenopathy. The heart is not enlarged. There is not significant pericardial effusion. The adrenal glands are not enlarged. There is a focal 3-cm mass lesion involving the pancreatic tail. The lesion demonstrates slightly higher attenuation relative to the remaining pancreatic parenchyma. Central low attenuation is noted, suggestive of cystic components. No significant surrounding inflammation is identified. There is not pancreatic ductal dilatation.

I have been reading as much as I can to try and understand what this means. Originally, when I started research, I had not began with the idea that all of these are tied to his rheumatoid arthritis; however, the more I read, a correlation seems to be drawn between many of these conditions and either rheumatoid arthritis, or prolonged exposure to methotrexate.

I had read one of the posts on the pulmonaryroundtable.com site title BAL eosinophilia in a patient with rheumatoid arthritis which seemed to resemble some of my husband’s case.

I wanted to ask what you thought of these results and to see if you thought that my supposition / correlations drawn in my own mind are simply that (suppositions on my part), or if you think that these links are founded, possible, and real.

I am also concerned about the noted lesion on the pancreas, as I do not seem to be able to find much regarding pancreatic mass lesions. It is very confusing reading

RADS

A 70 year old man has reactive airways dysfunction syndrome from a previous inhalant. He was seen in 2005 for dyspnea but primary did not initiate any steroids. At that time his fev1 was 2.05, 66% predicted. When we saw him in January where there was obstruction with an FEV1 of 2.34 (72% predicted). 20 mg of steroids were given (higher doses resulted in his fingers "locking") and a repeat spiro this month shows no change in the obstruction (2.27 l, 69% predicted).

How many would continue the steroids? How many would stop the steroids for apparent lack of efficacy?
Symptomatically he has mild dyspnea with exertion that is less than prior to the steroids.

Vasoreactivity testing in PH

Two cases today (pop an ibuprofen and read on...)

This comes to us from "IS"

After a R heart cath confirms pulm HTN and a vasodilator test with IV Flolan or inhaled NO confirms a responder, what do you guys start with? Do you start with calcium channel blocker sustained release or short acting? Do you leave the right heart cath in and monitor the hemodynamics as you titrate the dose of CCB? Just wondering as we are trying to start a PH program here and it seems that there isn't a standardized protocol. Thanks for your input.

Mediastinal adenopathy, hypoxia

Dr.Carrillo submitted a case for your enjoyment/pontification:

49yo Hx of ETOH and presumptive cirrhosis. Exposed to birds through his early years. 2000 had abnormal CT thorax with interlobular septal thickening, Pleural thickening encasing the lungs and multiple adenopathy in the med. Mediatinoscopy report says multiple venous collaterals may be early SVC.Thickened fat through the mediastinum.Bx of LN neg,no Cx sent. 2003 - Same CT findings.

Now presents with hypoxia and CT findings similar as before. The radiologist describes that the mediatinum looks abnormal with strading. I think this guy probably has histo with some fibrosis of the medistinum. I wonder if he has compression of the thoracic duct with causing his lymphatics to be prominent and show like interlobular septal thickening on CT and he had previous Chylo?. I was going to do broncoscopy with TBBX and TBNA and serologies. Any other idea or opinion about this case. Do you think I should further investigate the possible thoracic duct obstruction?

2-year-old mass

This is a 67 y/o male with a 75 p/y Hx of TOB but still good lung function (his FEV1 is 2.06L).
2 years ago he was told he had a "quarter-sized" nodule on his RML (at a different facility so I don't have those films) and he decided not to show up for his scheduled Bx or further follow-up visits. The lesion has clearly grown and I'd be surprised if it is anything other than a malignancy. Does the time of "follow-up" with growth but no spread in 2 years change your w/up sequence?
For example, if the PET shows no other disease, would you consider going straight to resection without a Bx?

Pulmonary HTN question

case comments: This is just a question: Are most of you still routinely performing a right heart catheterization prior to treatment initiation in patients with pulmonary hypertension - even in those associated with collagen vascular disease, etc? What do you use as your drug of choice for vasodilatory testing? Do you routinely perform a left heart cath as well?