Familial Mediterranean Fever Refractory to Colchicine: What Can the Hospitalist Do?

1Cleveland Clinic, Cleveland, OH

Meeting: Hospital Medicine 2011, May 10-13, Dallas, Texas.

Abstract number: 237

Case Presentation:

This is a 35‐year‐old Hispanic woman with a history of familial Mediterranean fever (FMF) on disability for 6 years given the severity and frequency of the FMF attacks. The patient had been unable to tolerate colchi‐cine because of severe gastrointestinal side effects. Dosage and frequency changes as well as implementing a lactose free diet had not improved these side effects. At this time, she was admitted with severe abdominal pain, generalized arthralgias, and low‐grade fever (99°F), as well as nausea, vomiting and nondysenteriform diarrhea. On review of symptoms, the patient complained of involuntary weight loss of 20 pounds in 14 months. History was significant for FMF in her paternal grandfather. Exam was remarkable for diffuse abdominal tenderness and guarding, but no rebound. The patient had multiple trigger points and had mild swelling and tenderness of both hands. Laboratory examination was negative or normal for ANA, anti‐Smith antibody, anti‐DNAds, anti‐CCP, rheumatoid factor, SPEP, UPEP, beta‐2‐microglobulin, lactate dehydrogenase, lactate, fibrinogen, and ferritin. No evidence suggestive of amyloi‐dosis was found. Computed tomography of the abdomen and pelvis was normal. The patient received intravenous volume resuscitation for rehydration. She received symptomatic treatment with ondansetron, morphine, and lopera‐mide. She was started on colchicine, with worsening of her abdominal symptoms even at a low dose of 0.3 mg/day. She had expressed intolerance to interferon in the past as well as to steroids. After discussion with rheumatology, she was started on anakinra 100 mg subcutaneously daily, with dramatic improvement of symptoms within 24 hours. The patient was discharged home asymptomatic with follow‐up by rheumatology as an outpatient.

Discussion:

FMF is manifested as recurrent attacks of fever associated with serositis as well as episodes of acute monoarticular arthritis. Long‐term complications such as amyloidosis can also occur. Attacks can last up to 5 days, with variable recurrence (every few weeks to every few years). Daily usage of colchicine results in cessation of attacks for most patients; however, 5%–10% of patients may be intolerant or refractory to colchicine. In some patients a lactose‐free as well as a gluten‐free diet may help to avoid the colchicine intolerance. Other alternative therapies include IFN‐alpha and methylprednisolone and drugs that block the actions of interleukin 1 (IL‐1). Currently 3 anti‐IL‐1 drugs are available. All are given as subcutaneous injections. Rilonacept is given weekly, anakinra daily, and canakinumab every 8 weeks. The use of IL‐1 pathway antagonists has extensive applications given the expanding knowledge of the role of IL‐1 in chronic inflammatory diseases.

Conclusions:

Hospitalists should be aware of novel therapies for inflammatory diseases that can be used as a rescue when conventional therapy fails. This case illustrates the importance of the knowledge of IL‐1 antagonists, which are becoming the flagship of anti‐inflammatory treatment.

Disclosures:

J. Barbastefano ‐ none; M. Auron ‐ none; S. Spalding ‐ none; A. Prabhakaran ‐none; V. J. Velez ‐ none

To cite this abstract:

Barbastefano J, Spalding S, Prabhakaran A, Velez V, Auron M. Familial Mediterranean Fever Refractory to Colchicine: What Can the Hospitalist Do?. Abstract published at Hospital Medicine 2011, May 10-13, Dallas, Texas. Abstract 237. Journal of Hospital Medicine. 2011; 6 (suppl 2). https://www.shmabstracts.com/abstract/familial-mediterranean-fever-refractory-to-colchicine-what-can-the-hospitalist-do/. Accessed July 23, 2019.

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