38. The Fours
The presentation had already started when Jax entered the auditory. I wanted to bury myself in the ground as he walked between chairs to sit next to me.
‘You’re late!’ I whispered mad at him.
Jax looked at his watch. ‘Five minutes, Liz, chill.’ He whispered back. ‘Even when I’m on time you think I’m late.’
The Medical Grand Rounds were always at 8am on Thursdays, so I always got to the auditorium by 7:45am, you know, just in case. Jax didn’t usually come for the Internal Medicine Grand Rounds, but that day Peter was going to present a case and he invited Jax.
Even from far, I could tell Peter was nervous. I didn’t know the case he was presenting, it was from a week we weren’t on the same floor, but I knew he was bothered by it.
‘So our patient, let’s call him Max, came to the ER last month for intense abdominal pain lasting 8 hours.’ Peter started. ‘Max was 55 years old. His pain was mainly epigastric and he had one episode of vomiting. He denied any other symptoms, but did say he had similar episodes of pain in the previous months, but nothing as severe. He had a previous history of hypothyroidism treated with a low dose of levothyroxine but used no other medications. He drank 2 beers on weekends and denied smoking. The only physical exam finding worth noting, besides the tender abdomen, was generalized lymphadenopathy.’’
He passed to the next slide which showed Max’s initial blood workup. There were a lot of mildly altered tests but his lipase stood out: over 4000.
‘Fortunately, as you can see, his labs were helpful in pointing the case towards pancreatitis, which was consistent with Max’s signs and symptoms, with the exception of his lymph nodes.’
Peter went on to tell us that Max initially responded well to treatment, improving his symptoms in the first days. The question was what caused the pancreatitis. An ultrasound showed no gallstones and all the routine investigation tests came back normal. Alcohol was becoming a strong candidate then, but it wasn’t fitting greatly. His GGT was normal and he didn’t seem to abuse alcohol, although he could be underestimating his drinking during history taking.
Then came the 4th day, when Max got worse. His pain came back so strong that he couldn’t even eat. So an abdominal CT was ordered.
‘To our surprise.’ Peter said when the CT images showed up on the screen. ‘His CT showed extensive fibrosis in the retroperitoneum, besides a swollen pancreas and no signs of acute complications. There was also a mass in one of his kidneys.’
‘He has 3 out of 4.’ I said to myself.
‘What?’ Jax asked me quietly.
‘You know, pancreas, retroperitoneum and a history of thyroid disease.’
‘So what?’ He asked when the person in front of us shushed us before I could answer.
Peter continued the case. Max’s pain medication was adjusted and he started improving a bit. His retroperitoneal fibrosis could be simply idiopathic, but I wasn’t buying it. His presentation was reminding me of an organizer I had made when I was in medical school - The Fours of IgG4. IgG4-related disease was a rare disease with a bunch of different and apparently unrelated conditions. I could never see it when it came up on a question, so I developed a way to memorize it.
I took a piece of paper from my backpack and drew it for Jax. It started with a little stickman. I circled his neck with number 1 and wrote thyroiditis, especially Riedel’s. Then I drew a number 2 on his chest, which meant noninfectious aortitis. In the epigastrium, I drew the 3 for autoimmune pancreatitis. And at last, a 4 in the lower abdomen to represent the retroperitoneal fibrosis. ‘The Fours of IgG4’ I wrote as a title while Jax looked at it interestingly.
Of course, IgG4 has several other related conditions, and actually having all of them can be incredibly rare, but that silly drawing helped. Anyway, Max seemed to have already 3 of those 4, so this diagnosis came to mind. And not only to mine.
‘The retroperitoneal fibrosis along with pancreatitis made us think about the possibility of a rare condition.’ Peter clicked on to the next slide. ‘IgG4-related disease. Which could also possibly explain his previous thyroid disease and lymphadenopathy.’
‘Not bad, nerd.’ Jax whispered to me.
‘So we ordered Max’s serum IgG4 concentration, which came greater than five times the upper limit of normal.’
I smiled to myself. That was such an interesting case, and Peter was presenting it brilliantly. I couldn’t see why he was so nervous about it. Yet.
They started Max on steroids promptly since his condition had been worsening. They also biopsied the kidney mass that was discovered on the CT to confirm the diagnosis. Indeed, the biopsy was unequivocal: Max had IgG4 disease.
It wasn’t until the very end that I saw it. A couple of days into the steroids treatment, Max had severe chest pain and altered mental status. Aortic dissection. Despite prompt and adequate management, he didn’t make it. And that explained why Peter was so off.
After he finished, a round of applause filled the room. He thanked the audience with a nod. Me and Jax walked up to him to say hi.
‘Congrats, Pete.’ Jax patted Peter on his back and Peter gave a sad smile back. ‘See you all later.’ He said and left in a hurry for the Peds rounds.
‘Hey, that was an incredible case.’ I said shyly.
Peter shrugged. ‘Max is dead.’
‘I know.’ I sat beside him on the stage. ‘It’s the worst part of the job.’ He nodded. ‘It’s just so frustrating. And there’s nothing I can do about it.’ ‘You can keep going.’ I got up and gave him a hand to do the same. ‘It’s the best thing we can all do.’
…
Want to know more about IgG4-Related Disease?
https://www.rarediseasesjournal.com/articles/igg4-related-disease-a-minireview.html
Want to read a real case of IgG4-Related Disease?
https://link.springer.com/article/10.1007/s13317-015-0069-3
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