VEXAS Syndrome (2)

"...You son of a bitch."

The curse tore out of me on its own. Strength drained from my entire body.

This was reality.

I was nothing more than some asshole writing fiction without even knowing the basics.

"...Ha. Fuck."

A hollow laugh burst out of me. Right. They say when something is so absurd you just start laughing.

A lunatic who'd regurgitated a case report? An asshole writing fiction?

My mind went ice-cold.

Wait. What if that bastard was right?

Everyone in the hospital knew Nam Seunghyun had the personality of a kicked hornet's nest, but he was a third-year Internal Medicine resident. The depth of experience he'd built up lived in a different league from a first-year like me, spinning my wheels in the ER. In his eyes, I might genuinely have looked like some idiot showing off half-baked knowledge I'd picked up off the floor.

What if this wasn't VEXAS at all? What if that Dead Doctors Gallery was just a delusion my brain had cooked ‍‍‌‌⁠​​⁠‌​​‍​​‍​‌​⁠‍‌‍⁠⁠‌⁠‌‍‌‍‌‌‌‍⁠​‌​‌​‌‍‌‌‌⁠‍​​‌‍⁠‌‌⁠⁠up, and it had only seemed to fit by coincidence? Then I was sitting here clinging to a disease that didn't even exist, having just gotten thoroughly, publicly destroyed by a third-year internist.

"Bullshit..."

Alright. Calm down first. Don't lead with the cursing. Think rationally. You're a doctor, for fuck's sake — not some shaman diagnosing by gut feeling.

Evidence. Proof. Evidence.

That was all that mattered.

I grabbed the bundle of the patient's previous hospital records that had been dumped in the corner of the On-Call Room. It was a wad of low-resolution PDF files, barely linked into our hospital's EMR as scanned copies.

"Let's see here, you pieces of shit."

"They dumped antibiotics into him and the fever still wouldn't break... Infectious causes are unlikely."

I scrolled down. Lab results packed the screen in dense rows. Hmm. Wait.

"...Huh?"

I narrowed my eyes. When I'd first skimmed the chart, I'd filed it under mild anemia and moved on. Hb 11.8. But the MCV sitting next to it snagged my attention.

Fuck. The upper limit of normal is 100. This was clearly macrocytic anemia. Not just anemia.

The anemia of chronic disease that shows up in a typical inflammatory picture is almost always normocytic. This was macrocytic.

"I missed this? No — I didn't miss it. I just didn't know what it meant."

My heart started pounding again. As if possessed, I picked up my phone and opened UpToDate. This time I pulled up the diagnostic criteria for VEXAS syndrome.

Right. You'd need genetic testing to know that. Can't confirm it right now, so skip.

"...Near-universal ‍‍‌‌⁠​​⁠‌​​‍​​‍​‌​⁠‍‌‍⁠⁠‌⁠‌‍‌‍‌‌‌‍⁠​‌​‌​‌‍‌‌‌⁠‍​​‌‍⁠‌‌⁠⁠finding."

Found in almost every patient. Holy shit. The very first piece was locking into place perfectly.

I went back to the chart. Radiology reports. Chest PA, abdomen CT.

"Right. Imaging was clean, so everyone got lost. Not cancer, not pus."

The common causes of FUO — abscess, tuberculosis, malignancy — were being ruled out. What remained was rheumatic disease, or some other rare inflammatory condition.

Finally, I combed through the nursing notes with a fine-tooth comb. The answers often hide in the scrawled physician charting and the meticulous records the nurses keep.

"Holy fucking eureka!!!"

I slammed my fist down on the On-Call Room desk without even realizing it.

Auricular chondritis. Nasal chondritis. A skin rash suggestive of neutrophilic dermatosis. And macrocytic anemia. The words describing VEXAS on the UpToDate screen overlapped with the patient's records, letter for letter.

The bastard who'd left that comment had clocked it in one second. From just a few lines of patient info.

I'd just been cheating off the answer key, and even then I'd had to go through all this bullshit just to realize the answer was actually correct. Doubt turned into certainty.

Nam Seunghyun, you son of a bitch.

If I gave up now, I'd be the real idiot. Not the patient — a worthless doctor who flinched at hierarchy and convention and looked away from the right answer sitting right in front of him.

Fuck it. They can kiss my ass.

I picked up my phone again. If the front gate's blocked, you climb the damn wall.

Which department owns this? Autoinflammatory disease... that's Rheumatology's turf.

I'd already gotten shut down by the Internal Medicine resident, so there was only one play left, right? Go straight to a Rheumatology professor.

It was practically a suicide mission. A first-year skipping the chief, the residents, even the fellows and calling a professor directly was a frontal assault on the hospital hierarchy. But I didn't care. There was a patient in front of me who needed saving, and I had a way to save him in my head.

I found the name of the on-call Rheumatology professor.

Alright. Okay.

I considered going through the chief first, but he already looked swamped, so I dropped it. With a trembling hand, I hit the call button. My heart felt like it was about to leap out of my mouth.

A voice thick with exhaustion. I opened my mouth as calmly as I could.

"Professor, good evening. I'm truly sorry to call so late. This is Han Hyeon-jae, first-year Emergency Medicine."

"Emergency Medicine? What's this about?"

Confusion and a hint of irritation mixed in the voice on the other end.

"Well... there's a patient in the ER right now with a fever of unknown origin. He's been through multiple tests at other hospitals and they never found a cause... and in my assessment..."

I took another deep breath. The bridge behind me was already on fire.

"I suspect VEXAS syndrome, which is why I called."

Silence. This time the silence was heavier than anything Nam Seunghyun had given me, by a mile.

"...What? VEXAS? Did you contact the Internal Medicine on-call?"

His voice was shaking.

"Ah, yes... Dr. Nam Seunghyun told me to rule out the basics first, but the patient's findings match too closely. I think you may need to come see him yourself, Professor... Is there any lab work I should order before you get here?"

You lunatic. You actual lunatic.

What the hell had I just done. A first-year ER grunt had just bypassed a third-year who sat at the top of the food chain and called a professor directly. The second Choi Youngjoon dismissed what I'd said as nonsense, I'd be branded a clueless maniac in this hospital, and that would be the end of me.

I looked around. I could feel the nurses glancing at me. Their eyes held a mix of emotions. That guy's got some serious balls. He's about to get torn apart like a dog. This is gonna be good. That kind of look.

Five minutes passed. Then ten. It felt like ten years. I fidgeted with the patient's chart, staring anxiously down the hallway. Cold sweat ran down my back.

Had he written it off as a first-year's delusion and ignored me after all?

Every worst-case scenario was tearing through my head when the automatic doors at the ER entrance slid open, and a man walked in.

Late thirties, maybe early forties. A young professor radiating an aura that said, I am not one of you slaves. Choi Youngjoon, Clinical Assistant Professor of Rheumatology.

Professor Choi Youngjoon swept a glance around, then walked straight toward me. My heart dropped.

"Dr. Han Hyeon-jae?"

"Yes, Professor! That's me!"

I shot to my feet and bowed at a full ninety degrees.

Professor Choi barely acknowledged the greeting, snatching the chart right out of my hands. From the previous hospital's records to the notes I'd just scrawled.

"Where's the patient?"

"Over there, Professor."

Professor Choi approached the patient without a word. He pulled on gloves and began examining him himself. He flipped the patient's eyelids to check the conjunctiva, then carefully palpated the pinnae, checking for cartilage swelling and tenderness.

Was my diagnosis right? No — it wasn't even my diagnosis to begin with. It was a comment from a dead medical scholar whose face I'd never seen. What if none of this was real?

After examining the patient for a long while, Professor Choi straightened up.

"Hmm..."

"Dr. Han. VEXAS has only been properly defined for a few years. You keep up with the latest literature, I take it?"

My mind went blank.

Interest? I don't give a flying fuck. Latest literature my ass. I could barely remember what I ate yesterday.

But how was I supposed to say that all of this was thanks to some insane gallery that had appeared inside my head? After a year of training, my thoroughly socialized mouth spat out the correct answer on instinct.

"Yes, Professor! That's right! I've always been interested in rheumatic diseases, so I look up related papers whenever I have time! I firmly believe we should always be acquiring new knowledge!"

The flattery and lies poured out so fluently it gave even me the chills. Professor Choi Youngjoon listened, then let out a short, dry chuckle. Whether it was a scoff at a ridiculous little shit or encouragement for a promising one, I couldn't tell.

"Good. I like that attitude."

The looks aimed at me had changed, too. Before I knew it, Professor Choi was putting in admission orders and a battery of tests.

A first-year resident — an Emergency Medicine resident, no less — had pinpointed a rare rheumatologic disease, called in a professor, and gotten the patient admitted. It was the kind of incident that would become ER legend.

Having finished giving his orders, Professor Choi Youngjoon straightened his coat and turned back to me.

"Han Hyeon-jae, was it?"

"Yes, Professor."

With that, Professor Choi left the ER as coolly and suddenly as he'd arrived.

I stared blankly down the hallway. The tension drained from my body and my legs started shaking. Something hot was filling the hollow space in my chest.

Now ‍‍‌‌⁠​​⁠‌​​‍​​‍​‌​⁠‍‌‍⁠⁠‌⁠‌‍‌‍‌‌‌‍⁠​‌​‌​‌‍‌‌‌⁠‍​​‌‍⁠‌‌⁠⁠it was time to figure out how to properly exploit these lunatics.