Effectiveness of cyclosporine as a treatment for steroid-resistant Cronkhite-Canada syndrome; two case reports

Yamakawa, K.; Yoshino, Takayuki; Watanabe, Kotaro; Kawano, Kenji; Kurita, Akira; Matsuzaki, Naomi; Yuba, Yoshiaki; Yazumi, Shujiro

doi:10.1186/s12876-016-0541-1

Cited by 27 publications

(17 citation statements)

References 15 publications

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“…The disease is usually progressive and may present potentially fatal complications, such as malnutrition, gastrointestinal bleeding and infection, which often occur with a 5-year mortality rate of 50.0% [ 9 ], if the disease is not treated or if treatment is inadequate or delayed [ 4 ]. However, spontaneous regressions were observed in 5-10.0% of cases of the syndrome, regardless of treatment [ 8 ].…”

Section: Discussionmentioning

confidence: 99%

“…Steroids are considered the main support of medical treatment, although the recommended dose and the duration of its use vary widely in the literature, with no current gold standard [ 4 ]. However, the prognosis after treatment with steroids is unknown [ 9 ]. The total treatment period is also unknown; recommendations range from 6 to 12 months of combined therapy [ 8 ].…”

Section: Discussionmentioning

confidence: 99%

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A Case of Adolescent Cronkhite-Canada Syndrome

Faria¹,

Basaglia

Nogueira³

et al. 2018

Gastroenterol Res

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The Cronkhite-Canada syndrome (CCS) was first reported in 1955, since then more than 500 cases have been reported, indicating an estimated incidence of one case per 1 million inhabitants. The syndrome occurs predominantly in male, with a ratio of three males to two females. Because of the rarity of the syndrome, there are no evidence-based therapies and the treatments described include a combination of therapies, but none is consistently effective. Surgery is usually reserved for the treatment of complications. Herein, we present a case of adolescent CCS. The patient was a 15-year-old boy who presented with watery diarrhea with 20 episodes a day, vomiting and abdominal pain for 4 weeks, with a weight loss of 8.0 kg (15.0% of initial weight). Endoscopic examination revealed polyposis in the stomach, duodenum, and colon. CCS was diagnosed and the patient was treated with a combined corticosteroid and metronidazole. Followed up at 8 month after the diagnosis, the patient was asymptomatic.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

A Case of Adolescent Cronkhite-Canada Syndrome

Faria¹,

Basaglia

Nogueira³

et al. 2018

Gastroenterol Res

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“…Relapse was common with steroid tapering, and longterm maintenance therapy by azathioprine may decrease its recurrence [12]. In view of supports of the immunologic basis for CCS, cyclosporine and Infliximab have been used as a treatment for steroid-resistant CCS [13,14]. With appropriate treatment, abdominal symptoms were relieved within a few months, whereas polyp regression often required more than 6 months (averaging 248 d for gastric polyps and 238 d for colonic polyps) [7].…”

Section: Figure 3 A: Gastric Polyp With Cystic Hyperplastic Glands Amentioning

confidence: 99%

Cronkhite-Canada syndrome: A rare case report and literature review

Lee¹,

Yang²,

Wu³

et al. 2018

biomedicalresearch

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Cronkhite-Canada Syndrome (CCS) is a rare, non-hereditary disease characterized by gastrointestinal polyposis associated with diarrhea and epidermal manifestations, such as hyperpigmentation, alopecia, and onychodystrophy. The pathogenesis of CCS is still not well understood but it probably involved autoimmune mechanism. Herein, we here report a case of steroid-responsive CCS in a male patient whose polyps presented with IgG-4 positive plasma cells. Keywords:Cronkhite-Canada syndrome, Hyperpigmentation, Autoimmune mechanism.Accepted on June 25, 2018 Case Report A 66 y old male farmer with no medical history of any systemic diseases had a 2 y history of non-bloody watery diarrhea associated with weight loss (more than 10 kg within 6 months). He defecated 6 to 8 times daily. He received upper and lower endoscopic examination, abdominal computer tomography, and small bowel barium series in the other hospitals, but there was no definite diagnosis. Endoscopic examinations revealed polyposis in both the stomach and colon. Pathology of the polyps showed inflammatory change without malignant change. Clinical symptoms were not alleviated after long-term proton pump inhibitor and mesalazine therapy. He had no family history of polyposis, malignancy of gastrointestinal tract, and he did not have fever and abdominal pain.His vital signs were normal on examination. General skin hyperpigmentation and dystrophic nails were found (Figure 1), but no oral pigmentation. Laboratory results revealed low level of serum albumin (2.7 g/dL, range: 3.8-5.3 g/dL) and cholesterol (100 mg/dL, range: 122-200 mg/dL). The erythrocyte sedimentation rate was elevated (42 mm/h, range: <15 mm/h), and the C reaction protein slightly increased (0.847 mg/dL, range: <0.3 mg/dL). Serum IgG was within normal range, and anti-nuclear antibody was negative. Gastroesophageal endoscopy found diffuse mucosal erythematous gastric mucosa with fold thickening and numerous semi-pedunculaed and sessile polyps with variable size at the cardia, body, antrum, and duodenal bulb with the exception of esophagus. Ileo-colonoscopic examination showed multiple erythematous sessile and semi-pedunculated polyps with variable size from ileocecal valve, cecum, to the left side colon (Figures 2A and 2B). Multiple EMR and polypectomy from the gastric and colon polyps showed retention polyps with cystically dilated glands filled with mucus in an edematous and inflamed lamina propria mainly composed of plasma cells, neutrophils, and eosinophils. IgG4-positive plasma cells were seen with uneven distribution (Figure 3). Focally, the number of IgG4-positive plasma cells was up to 30 in one high power field. The IgG4 to IgG-positive cell number ratio was around 40%. Helicobacter pylori was negative. Combining the polyposis, ectodermal manifestations, and clinical symptoms, he was diagnosed with Crokhite-Canada syndrome. The patient was treated with daily oral prednisolone 30 mg per day and then tapered over 3 months. The frequency of diarrhea was reduced and he gradually ga...

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“…There is no consensus for appropriate dose and duration of glucocorticoid therapy [4, 14, 15]. Immunomodulators including azathioprine, calcineurin inhibitors, and cyclosporine have been tried with mixed success [8, 16, 17]. …”

Section: Introductionmentioning

confidence: 99%

Cronkhite-Canada Syndrome: Sustained Clinical Response with Anti-TNF Therapy

Taylor

Kelly

Loomes

2018

Case Reports in Medicine

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Cronkhite-Canada syndrome (CCS) is a rare, nonfamilial syndrome that occurs in the sixth to seventh decades of life. It is characterized by acquired gastrointestinal polyposis with an associated ectodermal triad, including alopecia, onchodystrophy, and hyperpigmentation. CCS is characteristically a progressive disease, with a high mortality rate despite medical interventions. Disease complications are typically secondary to severe malnutrition, malignancy, GI bleeding, and infection. CCS is believed secondary to immune dysregulation; however, the underlying etiology remains to be determined. Treatment for CCS is largely anecdotal, and randomized controlled therapeutic trials are lacking due to the rarity of the disease. Aggressive nutritional support in conjunction with immunosuppression has been used previously with inconsistent results. In this report, we describe the presentation and diagnosis of a case of CCS and report encouraging treatment response with anti-TNF therapy.

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Effectiveness of cyclosporine as a treatment for steroid-resistant Cronkhite-Canada syndrome; two case reports

Cited by 27 publications

References 15 publications

A Case of Adolescent Cronkhite-Canada Syndrome

A Case of Adolescent Cronkhite-Canada Syndrome

Cronkhite-Canada syndrome: A rare case report and literature review

Cronkhite-Canada Syndrome: Sustained Clinical Response with Anti-TNF Therapy

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