Rare Case of Sarcoidosis with IBD Demonstrates Complexity of Disease

Rare Case of Sarcoidosis with IBD Demonstrates Complexity of Disease

A rare case report of a woman with sarcoidosis in the liver and spleen, who developed signs of inflammatory bowel disease (IBD) during recovery, illustrates that sarcoidosis can occur in less than typical ways.

The study by researchers at the University of Bari Aldo Moro in Italy, titled “A case of sarcoidosis with isolated hepatosplenic onset and development of inflammatory bowel disease during recovery stage,” was published in the journal Autoimmunity Highlights.

A 68-year-old woman who had type 2 diabetes and thyroid insufficiency arrived at the hospital with stomach pain and skin lesions. Her abnormal laboratory values indicated inflammation among other problems.

An ultrasound revealed that her liver and spleen were mildly enlarged and her liver showed signs of fat deposits. A spleen scan revealed several abnormal areas, tested further with computed tomography (CT). In addition to the fatty liver, the examination confirmed the presence of spleen lesions.

The medical team excluded the possibility that her symptoms were caused by bacterial or viral infection. Further magnetic resonance imaging (MRI) suggested that a part of her bile duct was smaller than normal.

A month later, physicians noted a CT scan showing that a lesion had formed in her lung lymph nodes, but again, infection was excluded. Because her bile duct still seemed to be blocked, the team performed a liver biopsy, which finally revealed the basis of her condition.

The tissue contained non-caseating granulomatous lesions and epithelioid and giant cells typical of sarcoidosis. She was treated with methylprednisolone, and two months later her lung and spleen lesions had vanished. Signs of inflammation and the blocked bile duct had improved, and doctors tapered her steroid dose.

Six weeks later, however, she returned to the hospital with stomach pain and chronic diarrhea. Although a colonoscopy had shown her gut mucosa was normal during the initial examinations, parts of it were now heavily inflamed, resembling aspects of Crohn’s disease, an inflammatory bowel disease (IBD). In addition, the inflammation did not seem to be caused by sarcoidosis granulomas.

Doctors again increased the dose of methylprednisolone, and within a couple of days, her diarrhea had improved.

The team noted that cases where sarcoidosis starts in the spleen and liver are rare. Only four previously published reports describe such cases. The development of IBD in association with sarcoidosis is possibly even more rare, particularly since the gut inflammation resolved within days — a response to treatment rarely seen in other patients with IBD.

The researchers said they urge fellow physicians to consider atypical symptoms and “intestinal inflammatory involvement should be seriously considered when the patient experiences symptoms.”

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Magdalena is a writer with a passion for bridging the gap between the people performing research, and those who want or need to understand it. She writes about medical science and drug discovery. She holds an MS in Pharmaceutical Bioscience and a PhD — spanning the fields of psychiatry, immunology, and neuropharmacology — from Karolinska Institutet in Sweden.

6 comments

    • Magdalena Kegel says:

      Hi Arthur,
      According to the study, sarcoidosis that starts in the spleen and/or liver, without affecting the lungs, is not so common.

  1. Sandra Smith says:

    This case study raises 2 issues that concern and confuse me about sarcoidosis:

    1) We assume that wherever we are looking now in the patient – or one step removed in this case – is where sarc “began”. How can we ever be sure where it really started, and what difference does it really make? Sarcoidosis is like a fire that never really goes out; we just keep treating the latest outbreak. Its genesis seems inconsequential, especially for those of us who are tx-resistant.

    2) I thought sarcoidosis was a disease whose end stage is scar tissue. Scar tissue never just disappears. So how did this patient’s lesions simply vanish after tx? The cells were already identified as giant-celled noncaseating granulomas, definitively sarcoid. Lesions, yes, but sarcoid lesions. How does that dead scar tissue revert back to being flesh that is now no longer detectable as a lesion?

    • Magdalena Kegel says:

      Hi Sandra,

      Thank you for your very relevant questions. I am not a medical doctor, and in no way a sarcoidosis expert, but I will try to answer your questions best I can.

      Regarding your first question, there is, of course, always a chance that doctors miss a sarcoid lesion that might be the primary lesion. At this point, reports of where sarcoidosis first emerged largely serve to gather research data to better understand the disease. The rarity of the condition makes case reports like this important to get a picture of different variants of the disease, which might, ultimately, provide insights into the causes and potential treatments of sarcoidosis.

      In your second question, you correctly point out that the end stage of a sarcoid lesion is scar tissue. But before reaching there, a lesion is made up of a certain type of inflammation. In many cases, this inflammation can be treated. In about half of patients, it goes into remission, meaning the sarcoid lesions are no longer detectable. Some people only have one sarcoidosis episode in their life, while many relapse, or have disease that does not respond to treatment. It might be so that these differences are caused by slightly different immune processes, but at this point, researchers simply don’t know why some people get better while others remain ill or relapse time after time.

      But if the inflammation persists and the tissue starts becoming fibrotic, the tissue will not become normal again, just as you pointed out.

      I hope that this answers your questions.

      • Sandra Smith says:

        Thank you very much for your response. You took the time to understand my questions. Plus, you took time to explain the “lesion life cycle”, which I know very little about. Much appreciated.

  2. Jennifer says:

    This is me! My sarc is in my spleen and liver. It grows and goes to my bone marrow and vertebrae and affects my red blood cells and entire GI system if I’m not on 20mg of Prednisone. I’ve found that I am different from most people with this disease which is rather frustrating.

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