My name is Dr Stephen Paget. I'm the physician-in-chief emeritus at Hospital for Special Surgery and in the Division of Rheumatology.
I'd like to tell you about a patient I saw about 2 months ago. This was a 43-year-old woman who had a 5-year illness that is really interesting. Five years ago, it started with diarrhea and abdominal cramps. She was seen by a gastroenterologist and eventually had a colonoscopy, which showed some mild local inflammation, but no definitive diagnosis of inflammatory bowel disease was made.
About a year ago, she was skiing in Switzerland and developed acute and severe incapacitating low back pain, to the point where she had to be brought off of the slopes and to a local hospital, where she was given hydration and eventually antibiotics. She had a normal sedimentation rate and complete blood count (CBC), a normal C-reactive protein, but severe abdominal pain. A CT scan of the abdomen with contrast showed a large retroperitoneal mass that was encasing the inferior vena cava and the aorta as well as other vessels coming off of the aorta and involving the ureters.
She was eventually transferred back to New York, where she was seen by a rheumatologist and then eventually by a kidney specialist because of the ureter involvement and an elevation in her serum creatinine. A biopsy was planned; they did another CT scan, which was going to guide the biopsy, and they found that the lesion was no longer big enough to biopsy. Interestingly enough, in a recent CT scan, not only did she have the inflammatory changes that I mentioned, albeit less intense than in Switzerland, but there was also some local inflammation in the bowel.
When I saw her, she was quite incapacitated with continued low back pain. She had 4+ out of 5 proximal muscle weakness in the arms and legs with normal distal strength, no synovitis, and no skin rash. She had back tenderness in the flank areas. She had a strongly positive antinuclear antibody (ANA) and very positive rheumatoid factor (RF). My feeling was that she clearly had a systemic problem, most probably retroperitoneal fibrosis or inflammation.
Her immunoglobulin (Ig)G4 level was normal, but she did have these other positive serologies, and she was about to go to Johns Hopkins to a retroperitoneal fibrosis expert. She wanted to go there before any treatment, but she traveled back to England, where she is from, and where she notified me that she had a recurrence of her severe abdominal pain. Under the circumstances, I put her on prednisone 40 mg, which resulted in complete disappearance of all of her back and abdominal pain, diarrhea, and fatigue.
She then went down to see this expert at Johns Hopkins who felt that she did not have retroperitoneal fibrosis mainly because the imaging studies did not show fibrosis, which obviously is necessary for a diagnosis of retroperitoneal fibrosis. However, all rheumatologists know that retroperitoneal inflammation can occur in all of our diseases, such as in systemic lupus erythematosus, Wegener granulomatosis, and other types of vasculitis. The scarring can occur particularly in the setting of IgG4 disease, which is a scarring-type disorder.
She then came back to me. Yesterday, she was down to prednisone 15 mg daily, and she is doing quite well. Her sedimentation rate is normal. Her C-reactive protein is normal, as is her CBC. Her RF continues to be strongly positive, and she is negative for anti-cyclic citrullinated peptide antibodies. She has a positive ANA of 1 to 640, which is a speckled pattern, and she is also positive for anti-Saccharomyces cerevisiae antibody, which tends to be positive in inflammatory bowel disease.
One interesting historical point is that when she was having severe back pain, the pain would go away if she had a bowel movement. Clearly, there was an interconnection in the development of her inflammatory bowel symptoms, even though the colonoscopy was negative, and her retroperitoneal mass.
At this time, I've started her on methotrexate as a steroid-sparing agent, and I'm starting to taper her prednisone. Hopefully, her disease will now be suppressed.
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Cite this: Low Back Pain and GI Symptoms on the Swiss Alps - Medscape - Aug 26, 2015.