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Efficacy of Adalimumab in a Girl with Refractory Intestinal Behcet's Disease.

Kaji M, Kishi T, Miyamae T, Nagata S, Yamanaka H, Fujikawa S - Case Rep Rheumatol (2015)

Bottom Line: Methylprednisolone pulse therapy was initiated, after which the symptoms transiently improved, but, during the corticosteroid taper, the abdominal pain recurred.Of importance, the dose of corticosteroids was successfully reduced without exacerbation during 8 months of observation.This is the first reported case in which adalimumab was used for pediatric gastrointestinal Behcet's disease.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatrics, Tokyo Women's Medical University, No. 8-1, Kawadacho, Shinjuku-ku, Tokyo 162-0054, Japan.

ABSTRACT
We describe our experience with a juvenile patient who had refractory intestinal Behcet's disease that responded to adalimumab, a fully humanized antibody against soluble TNF-α and its receptor. The patient, a 13-year-old girl, presented with oral aphthous ulcers, vulvar pain, and rashes on the lower extremities. She gradually developed a low-grade fever, abdominal pain, diarrhea, and hematochezia. Lower gastrointestinal endoscopy revealed ulcers in the terminal ileum, consistent with intestinal Behcet's disease. Methylprednisolone pulse therapy was initiated, after which the symptoms transiently improved, but, during the corticosteroid taper, the abdominal pain recurred. The symptoms resolved soon after the administration of adalimumab. Of importance, the dose of corticosteroids was successfully reduced without exacerbation during 8 months of observation. This is the first reported case in which adalimumab was used for pediatric gastrointestinal Behcet's disease. Adalimumab is a good choice for intestinal Behcet's disease refractory to conventional treatment.

No MeSH data available.


Related in: MedlinePlus

Lower gastrointestinal endoscopic observations. (a) On admission, lower gastrointestinal endoscopy revealed ulcers in the terminal ileum. (b) A week after the initiation of adalimumab, lower gastroendoscopy showed epithelialization of the ulcers.
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fig3: Lower gastrointestinal endoscopic observations. (a) On admission, lower gastrointestinal endoscopy revealed ulcers in the terminal ileum. (b) A week after the initiation of adalimumab, lower gastroendoscopy showed epithelialization of the ulcers.

Mentions: The patient, a 13-year-old girl, had a second-generation Korean father, a Japanese mother, an elder brother, and a younger sister. There was no family history of autoimmune disease. The patient was admitted to our hospital for acute tonsillitis a year before the current admission. She had oral aphthosis recurring more than three times in 12-month period and repeated genital aphthosis. The patient sought evaluation at our hospital for oral ulcers, vulvar pain, headaches, arthralgias, and erythema on the lower extremities in June 2013. She was admitted to the dermatology department. Following the gradual onset of abdominal pain and bloody diarrhea (Figure 1), she was referred to the pediatric department. On admission, the patient had a temperature of 36.2°C, a heart rate of 81 beats per minute, and a blood pressure of 130/86 mmHg. At the time of admission, her physical findings were appropriate for her age, but she lost 2.2 kg during the first week while being admitted to the dermatology department. Edematous erythema and erythema-nodosum-like eruptions were noted on the lower extremities (Figure 2). There were painful ulcers on the left buccal mucosa and left labia minora. Blood testing revealed a significant elevation of the white blood cell count (14.17 × 103/μL) and C-reactive protein (CRP; 4.29 mg/dL), fibrinogen degradation products (FDP; 27.9 μg/mL [nl < 4.0]), D-dimer (15.60 μg/mL [nl < 1.0]), and IgD levels (27.5 mg/dL [nl < 9.0]). Human leucocyte antigen (HLA)-B51 was positive. An abdominal CT showed a thickened colonic wall. Lower gastrointestinal endoscopy revealed ulcers in the terminal ileum (Figure 3(a)) and biopsies of the ileum revealed nonspecific chronic inflammation. The ophthalmologic examination did not show the presence of uveitis. Pathergy testing was negative. Histology of the skin lesions on the lower extremities revealed neutrophilic dermatitis in the dermis. The symptoms met the criteria established by the International Study Group for the diagnosis of Behcet's disease [4, 5] with gastrointestinal manifestations.


Efficacy of Adalimumab in a Girl with Refractory Intestinal Behcet's Disease.

Kaji M, Kishi T, Miyamae T, Nagata S, Yamanaka H, Fujikawa S - Case Rep Rheumatol (2015)

Lower gastrointestinal endoscopic observations. (a) On admission, lower gastrointestinal endoscopy revealed ulcers in the terminal ileum. (b) A week after the initiation of adalimumab, lower gastroendoscopy showed epithelialization of the ulcers.
© Copyright Policy - open-access
Related In: Results  -  Collection

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getmorefigures.php?uid=PMC4644826&req=5

fig3: Lower gastrointestinal endoscopic observations. (a) On admission, lower gastrointestinal endoscopy revealed ulcers in the terminal ileum. (b) A week after the initiation of adalimumab, lower gastroendoscopy showed epithelialization of the ulcers.
Mentions: The patient, a 13-year-old girl, had a second-generation Korean father, a Japanese mother, an elder brother, and a younger sister. There was no family history of autoimmune disease. The patient was admitted to our hospital for acute tonsillitis a year before the current admission. She had oral aphthosis recurring more than three times in 12-month period and repeated genital aphthosis. The patient sought evaluation at our hospital for oral ulcers, vulvar pain, headaches, arthralgias, and erythema on the lower extremities in June 2013. She was admitted to the dermatology department. Following the gradual onset of abdominal pain and bloody diarrhea (Figure 1), she was referred to the pediatric department. On admission, the patient had a temperature of 36.2°C, a heart rate of 81 beats per minute, and a blood pressure of 130/86 mmHg. At the time of admission, her physical findings were appropriate for her age, but she lost 2.2 kg during the first week while being admitted to the dermatology department. Edematous erythema and erythema-nodosum-like eruptions were noted on the lower extremities (Figure 2). There were painful ulcers on the left buccal mucosa and left labia minora. Blood testing revealed a significant elevation of the white blood cell count (14.17 × 103/μL) and C-reactive protein (CRP; 4.29 mg/dL), fibrinogen degradation products (FDP; 27.9 μg/mL [nl < 4.0]), D-dimer (15.60 μg/mL [nl < 1.0]), and IgD levels (27.5 mg/dL [nl < 9.0]). Human leucocyte antigen (HLA)-B51 was positive. An abdominal CT showed a thickened colonic wall. Lower gastrointestinal endoscopy revealed ulcers in the terminal ileum (Figure 3(a)) and biopsies of the ileum revealed nonspecific chronic inflammation. The ophthalmologic examination did not show the presence of uveitis. Pathergy testing was negative. Histology of the skin lesions on the lower extremities revealed neutrophilic dermatitis in the dermis. The symptoms met the criteria established by the International Study Group for the diagnosis of Behcet's disease [4, 5] with gastrointestinal manifestations.

Bottom Line: Methylprednisolone pulse therapy was initiated, after which the symptoms transiently improved, but, during the corticosteroid taper, the abdominal pain recurred.Of importance, the dose of corticosteroids was successfully reduced without exacerbation during 8 months of observation.This is the first reported case in which adalimumab was used for pediatric gastrointestinal Behcet's disease.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatrics, Tokyo Women's Medical University, No. 8-1, Kawadacho, Shinjuku-ku, Tokyo 162-0054, Japan.

ABSTRACT
We describe our experience with a juvenile patient who had refractory intestinal Behcet's disease that responded to adalimumab, a fully humanized antibody against soluble TNF-α and its receptor. The patient, a 13-year-old girl, presented with oral aphthous ulcers, vulvar pain, and rashes on the lower extremities. She gradually developed a low-grade fever, abdominal pain, diarrhea, and hematochezia. Lower gastrointestinal endoscopy revealed ulcers in the terminal ileum, consistent with intestinal Behcet's disease. Methylprednisolone pulse therapy was initiated, after which the symptoms transiently improved, but, during the corticosteroid taper, the abdominal pain recurred. The symptoms resolved soon after the administration of adalimumab. Of importance, the dose of corticosteroids was successfully reduced without exacerbation during 8 months of observation. This is the first reported case in which adalimumab was used for pediatric gastrointestinal Behcet's disease. Adalimumab is a good choice for intestinal Behcet's disease refractory to conventional treatment.

No MeSH data available.


Related in: MedlinePlus