Chapter 2
“I’ll explain it slowly, first.”
I have a duty to properly explain why this disease matches that patient’s current condition.
“I found it while reading a case report... It seemed like that rare disease.”
[Yeah. Go on.]
I let out a long sigh and opened UpToDate and other disease information sites.
Rationally.
I’m a doctor.
Not some shaman who diagnoses by gut feeling.
Evidence.
Proof.
Evidence!
That was all there was to it.
“Let’s see here, records.”
Treated at a local clinic with symptomatic care (*treatment aimed at symptom relief) for a cold and body aches, but no improvement
Admitted to a local secondary hospital with chief complaints of severe generalized fatigue, multiple joint pain and muscle pain, and weight loss over three months....
I scrolled down.
The page was packed with all kinds of lab results.
“At first, the white blood cell count was at the lower limit of normal, platelets were normal too, but hemoglobin had dropped to 8.2....”
Vitamin B12, folate, and thyroid function tests checked at the same time were all normal.
“The secondary hospital must have suspected an infection at first.”
[Yeah. Probably. But I’m a little busy right now. Let’s talk later.]
“Ah, uh, okay.”
All the better for me.
That gave me time to reason it out properly.
CRP(* C-reactive protein), ESR(* erythrocyte sedimentation rate), and ferritin were all markers of severe inflammation.
Hmm.
‘The secondary hospital gave antibiotics.’
There was a record of administering third-generation cephalosporins and macrolides with pneumonia and sepsis in mind.
Microbial cultures and a respiratory virus panel were also done, but....
The results showed nothing.
Even the high fever and respiratory symptoms didn’t improve.
‘The steroids worked, and when the dose was reduced, it relapsed, so that’s how they ended up at our hospital.’
Click-
I opened UpToDate again.
The diagnostic criteria for ‘VEXAS syndrome.’
Somatic UBA1 gene mutation in myeloid cells (*mutation of the UBA1 gene in myeloid cells)
Hmm, this would only be known through genetic testing.
I couldn’t confirm that right now, so I skipped it.
‘Anemia is found in nearly all cases. Progressive thrombocytopenia may also be observed’....
“…Near-universal finding.”
It was found in almost every patient.
Check, for now.
The patient is anemic.
I went back to the chart.
‘There was severe bilateral periorbital edema.’
And the patient showed a cytokine-mediated inflammatory state.
So they gave steroids....
‘So the condition improved again?’
But the hemoglobin level was still stuck at 8.4.
‘And the macrocytosis still hadn’t been corrected at all.’
Click-
Eye pain discovered on hospital day 15. Ophthalmology consult: episcleritis.
Click-
Right auricular chondritis.
Click-
Skin rash suspicious for neutrophilic dermatosis.
And macrocytic anemia.
The letters for VEXAS on the UpToDate screen overlapped the patient’s records exactly, letter by letter.
‘The anemia got worse again. At this rate....’
If it kept relapsing this often even on a maintenance dose of over 10 mg of prednisolone, and was highly steroid-dependent, then it had to be something different from a typical autoimmune disease.
Wait, why am I looking at someone else’s patient like this?
Anyway.
‘This looks like VEXAS.’
The bastard who left that comment recognized it in one second.
Just from a few lines of patient information.
I’d only copied off that answer sheet, and yet I still had to go through all this bullshit to realize the answer was right.
Doubt turned into certainty.
‘Right.’
I picked up my phone again.
…
**
…
Once the information had been relayed, I could finally relax after hearing Kang Taejin answer in a cheerful voice.
Hoo....
I’d decided to save my deductions about this gallery for when I got home, and I was waiting for Kang Taejin in front of the elevator on the floor where the hospital’s internal medicine office was located.
Kang Taejin shuffled out, dragging his feet.
“Hyeonjae.”
“What is it?”
“I got shot down.”
…?
What?
“You got shot down?”
“The third-year, Dr. Nam Seunghyeon, was asking if I’d seen something weird somewhere or what. I laid out my reasoning, too... No, this looks right no matter how you look at it. The antibiotics don’t work, it’s heavily steroid-dependent, and recurrent polychondritis comes with macrocytic anemia. That’s VEXAS, plain and simple.”
I thought it over carefully and came to a conclusion.
“Taejin.”
“What.”
If the front gate was blocked, we’d just have to climb the wall.
‘Which department handles this? Autoinflammatory diseases... This is rheumatology.’
Since we got shut down by an internal medicine resident, there was only one way, wasn’t there?
Go straight to the rheumatology professor.
It was practically a suicide mission.
For a mere first-year resident to skip over the chief and fellow and call the professor directly was a head-on challenge to the hospital hierarchy.
But I didn’t care.
There was a patient who needed saving, and I already had a way to save that patient in my head.
I found the name of the rheumatology attending on call.
“Let’s call the professor.”
“What?”
“Let’s go straight to the professor.”
Kang Taejin was horrified by my suggestion.
“Hey, even so...”
“We’ve finally got a clue to the diagnosis of a patient who’s been hospitalized for weeks—are you really going to let it slip away?”
“Well... no, I guess not.”
I took a breath.
Hoo.
[Rheumatology Clinical Assistant Professor Choi Youngjun]
Deep breath, okay.
“Taejin.”
“Yeah?”
“You’re the one who’s going to explain it?”
“Ah, um... yeah. I’ll do it. I rotated through rheumatology first.”
I watched Kang Taejin pull out his phone.
Kang Taejin’s hands trembled as he pressed the call button.
[…Hello.]
A voice thick with exhaustion.
Kang Taejin opened his mouth calmly.
“Professor, good evening. I’m very sorry to call so late. I’m Kang Taejin, first-year in internal medicine.”
“A first-year? What is it?”
The voice on the other end sounded puzzled, with a hint of irritation mixed in.
“Um... I’m calling about patient number 26110134.”
[Wait. Say the medical record number again.]
“It’s patient 26110134.”
[So what about this patient?]
I mouthed V-E-X-A-S.
Say it.
Say it.
“In my opinion....”
Kang Taejin took another deep breath.
The bridge back was already burned.
Go straight ahead.
“I’m calling because I suspect VEXAS syndrome.”
Silence.
[……What? VEXAS? Did you report it to your senior first?]
The voice was trembling.
“Ah, yes... well, Dr. Nam Seunghyeon said to rule out the basics first, but the patient’s findings fit too well. I think you may need to come see the patient yourself.... Before that, is there any test order I should put in first?”
[I’m coming now. Wait there, Dr. Kang.]
Click-
The call ended.
Kang Taejin looked at me, trembling.
“Is this really okay?”
“Well... if we’re screwed, we’re screwed together. That’s camaraderie and patriotism for you.”
Crazy bastard. A total crazy bastard.
What the hell had I just done?
A mere first-year ER newbie had talked his internal medicine buddy into skipping over some sky-high third-year and calling the professor directly.
If Professor Choi Youngjun dismissed us as nonsense, I’d be branded in this field as nothing but a clueless lunatic and that would be the end of it.
I looked around.
I could feel several staff members glancing my way.
Their gazes were mixed with all sorts of emotions.
That guy’s got guts, huh?
He’s about to get chewed up like a dog. Now this is interesting.
That kind of look.
Five minutes passed.
Ten minutes passed.
It felt like ten years.
I fiddled with my phone for no reason and stared anxiously down the end of the hallway with Kang Taejin.
Cold sweat ran down my spine.
Had they decided it was just a first-year’s delusion and ignored it after all?
Just as all kinds of worst-case scenarios were tearing through my mind,
the elevator’s automatic doors slid open, and a man walked out.
A man who looked to be in his late thirties or early forties.
He was a young professor with an aura that said he was leagues above the rest.
Rheumatology Clinical Assistant Professor Choi Youngjun.
Professor Choi Youngjun glanced around once, then headed straight toward me.
My heart dropped.
“Dr. Kang Taejin?”
“Ah... I’m, uh, Han Hyeonjae from Emergency Medicine.”
“Oh. Dr. Kang Taejin?”
“I’m Kang Taejin, first-year in internal medicine!”
Kang Taejin shot me a sidelong glance.
Oh.
Was he telling me to run?
Nice sense of timing.
“Why’s the ER buddy here?”
The atmosphere got a little chilly.
Kang Taejin hesitated for a long moment before finally speaking.
“W-well... actually, this friend was the one who first said it might be VEXAS.”
Dr. Choi rolled his eyes and looked at my ID badge.
“…Dr. Han Hyeonjae?”
“Yes! Professor! I’m Han Hyeonjae!”
I bowed at ninety degrees.
Professor Choi Youngjun gave my greeting the barest acknowledgement and headed toward the inner ward station.
“What are you doing? Why aren’t you following, Dr. Han Hyeonjae?”
Oh, shit.
I’m not really the kind of person who should be going in there...
so I just followed him inside.
From the previous hospital’s records to the notes Kang Taejin had just scribbled after hearing me out, Professor Choi Youngjun began examining everything carefully.
Was my diagnosis right?
No, it wasn’t even my diagnosis to begin with.
It had just been a comment from some dead doctor I didn’t even know the face of.
What if none of this was right?
After examining the patient for a long while, Professor Choi straightened up.
“Hmm....”
“Dr. Han. VEXAS is a disease that’s only been properly defined for a few years. You must be interested in the latest findings, huh?”
My mind went blank for a moment.
Interested?
Not a damn bit.
Latest findings, my ass.
I can barely remember what I ate yesterday.
But how could I possibly say that all of this was thanks to some crazy gallery that had just appeared in my head?
My mouth, perfectly socialized through a year of training, instinctively spat out the right answer.
“Yes! That’s right, Professor! I’ve always been interested in rheumatologic diseases, so whenever I have time I look up related papers! Of course, I think we should always be learning new things!”
A torrent of flattery and lies came out so smoothly it gave even me goosebumps.
Professor Choi Youngjun heard my answer and let out a short snort of laughter.
Whether it was mockery of some ridiculous bastard or encouragement for a good one, I couldn’t tell.
“Good, I like that mindset.”
Before I knew it, Dr. Choi was already placing orders for all sorts of tests.
“Send out a bone marrow biopsy, Sanger sequencing (*DNA sequence analysis by the Sanger method), and NGS (*next-generation sequencing). Let’s do those.”
A first-year resident.
And not just any first-year resident, but an emergency medicine resident, had accurately pinpointed a rare rheumatologic disease and even gotten the professor to come and order the tests.
It was the stuff of ER legend.
After finishing all his instructions, Professor Choi Youngjun straightened his coat and looked back at me.
“You said your name was Han Hyeonjae, right.”
“Yes, Professor.”
“You did well for now. It’s hard to make a definitive diagnosis based on clinical symptoms alone, but... it fits. Refractory to conventional immunosuppressants, chondritis, histiocytic skin lesions, macrocytic anemia. From what I can see, the clinical picture fits VEXAS.”
With that final remark, Professor Choi left the ward just as coolly as he had arrived.
I stared blankly toward the end of the hallway.
The tension drained from my whole body, and my legs wobbled.
It felt like something hot was welling up in my empty chest.
[Dead Medical Scholar Gallery]
Now it was time to think about how to properly use these lunatics to my advantage.