A middle aged woman presents with many months of pleuritic chest pain and dyspnea. PFTs and CXR are shown (current CXR on right, prior from 5 yrs ago on left). DLCO is normal. HRCT shows no parenchymal lung disease. 1/
Which of the following co-morbid conditions might explain the abnormal CXR and PFT findings:
2/
Shrinking Lung Syndrome (SLS) is a rare complication of SLE or occasionally other #autoimmune/#rheumatologic conditions.
SLS is characterized by a triad of dyspnea, pleuritic chest pain, & progressive decrease in lung volumes w/o interstitial disease.
Here are her serologies 3/
First described in 1965, SLS is thought to be caused by repeated episodes of pleuritis causing limited muscle engagement and reduced inflation, leading to a progressive decrease in lung volumes and compliance.
4/
The prognosis of Shrinking Lung Syndrome (SLS) is good. Most patients respond favorably to a course of glucocorticoids (typically 40-60mg prednisone daily), with lung function & imaging often returning (almost) to baseline.
Well illustrated by this case: 5/
To summarize what we’ve learned about SLS:
* occurs in <1% of pts with SLE (rarely other rheum conditions)
* characterized by pleuritic chest pain, dyspnea, & decreased lung volumes w/ normal parenchyma
* due to repeated episodes of pleuritis
* is usually steroid responsive
6/
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77 yo with respiratory distress, RR 30, SpO2 80% on non-rebreather at 15 lpm
CXR & TTE are unrevealing
pH 7.58 / PaCO2 24 / PaO2 >500 / HCO3 22
MetHb 0% CarboxyHb 0%
The ABG looks like this:
The answer is sulfhemoglobinemia.
Sulfhemoglobinemia is a *permanently* modified hemoglobin associated with exposure to TMP/SMX, dapsone, phenazopyridine, & other amino & nitro compounds.
It has an altered oxy-hemoglobin dissociation curve.
2/
Sulfhemoglobinemia is easily confused with methemoglobinemia. Both have very dark colored blood & present with cyanosis. Diagnosis typically requires a specialized lab.
Spoiler: you may have heard that SulfHb is green. It isn’t really. You’re thinking of Vulcans’ blood.
Damn. Under Trump the White House Medical Unit was a pill-mill. Thousands of ambien & provigil per month.
Worse, for a clinic that doesn’t typically do procedures w/ moderate sedation they sure are they ordering prodigious quantities of morphine, fentanyl, versed, & ketamine…?
Honestly, this reminds me of Norman Ohler’s Blitzed.
The AG report was largely concerned with the enormous cost of prescribing these non-genetic meds.
It’s worth pointing out that dispensing prescription meds without documentation is malpractice. In the case of controlled substances it’s also likely a crime.
The long awaited #COVIDOUT RCT is now in @TheLancet:
- high risk adults randomized to either metformin (MET), ivermectin (IVM), fluvoxamine (FLV) or placebo.
- MET reduced the risk of long COVID (6.3% vs 10.4%; NNT = 24)
- no benefit with IVM or FLV