IgG4-RD Question of the Week: Were “Comfort Measures Only” Appropriate?

I received an email message last week from a resident in internal medicine that was both extremely moving (for reasons you will understand) and heartening, for reasons that I will explain. I share the story with permission.

The message

“I am a second-year resident physician in internal medicine. I am sorry to email you out of the blue, and I hope this is not too much of a bother, but I have a question regarding an interesting case of potential IgG4-RD that I saw recently. I was hoping to get your opinion on this case, simply for my own learning and curiosity.

This was the case of an 88-year-old gentleman who presented to our hospital with a 2-week history of profound fatigue, malaise, anorexia, and 10–15-pound weight loss. He was jaundiced and a CT scan of the abdomen revealed a large mass within the central intrahepatic biliary tree, resulting in marked dilatation of the common bile duct. The radiologic appearance was felt to be most consistent with a new diagnosis of cholangiocarcinoma (a cancer of the bile ducts).

When I assessed the patient for the first time, however, I discovered that he had a history of unexplained chronic pancreatitis as well as chronic kidney disease caused by retroperitoneal fibrosis. This history went back nearly 15 years, but it seemed that the findings had never been investigated deeply. It seemed to me, in the context of this new biliary mass, that IgG4-related disease needed to be considered as a cause of the gentleman’s overall presentation.

I therefore sent a blood sample for an IgG4 measurement and spoke with the interventional gastroenterologist, who favored the diagnosis of cholangiocarcinoma (a cancer of the bile ducts) given the size of the mass. Brushings of the common bile duct mass – not a full biopsy – were sent for cytopathology. While awaiting these investigations, conversations about palliative care were raised because of the patient’s advanced age and preferences.

The team decided, after long deliberation with the patient and his family, to offer comfort measures only, and the patient passed away shortly after. The IgG4 level returned after a couple of days and was found to be elevated to 330 mg/dL (more than four times the upper limit of normal). The brushings that had been done on the mass, less sensitive than a full biopsy, did not show cancer.

The reason I am emailing you about this case is because, in a way, it does not sit right with me that no active treatment was offered without clearly establishing a diagnosis (especially when a potentially treatable condition was a real possibility and now seems even more likely in retrospect). I had proposed to try a course of prednisone and repeat the imaging a short while later to see if there was a response of the mass to treatment. Obviously, a good response to treatment would have favored the diagnosis of IgG4-RD further. Ultimately, this approach was overruled, and the outcome was as reported.

I’m interested in knowing your thoughts. I realize there are no easy answers to these questions, so I thank you very much in advance for indulging me. I have always had a strong interest in immunologic and systemic auto-inflammatory diseases, and your work in this area is truly inspiring.”

The response

Thank you for writing and sharing this challenging case. I'm so impressed by your clinical intuition and, of course, the degree to which the patient’s plight and the difficult end-of-life decisions affected you.

I agree that this patient probably did have IgG4-RD, and if there was a reasonable expectation that he could return to a functional status he would have been happy with, then an empiric course of treatment with prednisone would have made good sense. Assuming he responded to a short initial course of prednisone, I would have offered him B cell depletion as a more definitive way of trying to induce remission.

I suspect the conversation would have been different if the patient had been 48, 58, 68, or even 78 years old. Without knowing more about the pre-illness health status of this patient, it's difficult for me to comment on the decision-making in this specific case, which may well have been appropriate considering the full picture of the gentleman’s overall condition, the likelihood of a meaningful recovery given the possibility of other health issues, and (of course) discussions with the patient and family, which I’m sure were quite emotional.

Thank you for continuing to try, and learn from the case and sharing. I'm delighted to hear of your interest in immunology and inflammatory disease. If you're interested in following IgG4-RD, please visit www.igg4ward.org, subscribe to the website, and join our online Physician Network. I am happy to discuss any aspect of this further.

My own reflections

Situations like this one provide some of our most poignant moments as physicians. At these times, we agonize with patients and their families about the right course. I’m saddened by the patient’s death, though it may well be that the outcome was inevitable given his advanced age and what may have been a very poor health status even before the diagnosis of IgG4-RD was considered.

Although this kind of thinking is “Monday morning quarterbacking,” we learn from such exercises to try and help the next patient. One can’t help considering what the patient’s health might have been like had the diagnosis of IgG4-RD been considered and made years earlier when he had both pancreatitis of a cause that could not be determined at the time and retroperitoneal fibrosis. Those conditions, plus the finding of a very elevated serum IgG4 concentration years later, make the diagnosis of IgG4-RD likely, and things might have been different had the diagnosis been established years earlier and proper treatment administered then.

I’m encouraged by one fact. IgG4-RD – probably the correct diagnosis in this case – was considered by a young physician even when the diagnosis probably did not enter the minds of his older colleagues. This is a sign that things are changing for people living with IgG4-RD (whether they know of their diagnosis yet or not). The next time, the diagnosis may be made earlier, and the outcome could be different.

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