Bowel perforations in a patient affected by Churg–Strauss syndrome under high-dose steroid treatment: will alternative drugs reduce risk of surgery?

Venditti, D; Ielpo, Benedetto; Buonomo, Oreste Claudio; Petrella, Giuseppe

doi:10.1007/s00296-009-1289-5

Cited by 15 publications

(16 citation statements)

References 8 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Eosinophilic granulomatosis with polyangiitis (EGPA) belongs to the systemic vasculitides which may develop life-threatening major organ involvement Any type of vasculitis has the potential to cause local or diffuse pathological changes in the gastrointestinal (GI) tract based on the inflammation of vessel walls, followed by alterations of the blood flow and ischemic damage to the dependent organ (Çileda» et al 2012; Singh et al 2009; Venditti et al 2011). Intestinal manifestations have to be considered more common in other vasculitis disease, such as polyarteriitis nodosa, however, it seems to occur as severe manifestation of EGPA (Nakamura et al 2002).…”

Section: Introductionmentioning

confidence: 99%

“…Several case reports have been published with EGPA and GI tract involvement. Venditti et al have previously published a case of EGPA vasculopathy with large bowel perforation, however, the patient has been under long-term treatment of GC during his course of disease (Venditti et al 2011). Two cases of EGPA have been presented with intestinal vasculitis, one of them with perforation as initial presentation of EGPA after 20 days of admission in the hospital (Venditti et al 2011; Murakami et al 2004).…”

Section: Introductionmentioning

confidence: 99%

“…Venditti et al have previously published a case of EGPA vasculopathy with large bowel perforation, however, the patient has been under long-term treatment of GC during his course of disease (Venditti et al 2011). Two cases of EGPA have been presented with intestinal vasculitis, one of them with perforation as initial presentation of EGPA after 20 days of admission in the hospital (Venditti et al 2011; Murakami et al 2004). Varbanova et al have presented a similar EGPA case to the cases, which here have been presented, showing vasculitic intestine perforation within 3 weeks after diagnosis of EGPA (Varbanova et al 2011).…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Gastrointestinal perforation due to vasculitis at primary diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA) despite a high dose glucocorticosteroids treatment

et al. 2014

View full text Add to dashboard Cite

BackgroundEosinophilic granulomatosis with polyangiitis (EGPA) belongs to the systemic ANCA-associated vasculitides which may develop life-threatening major organ involvement, such as eosinophilic pulmonary infiltration, neuropathy, acute nephritis, myocarditis, and gastrointestinal (GI) tract involvement. Here, two cases of EGPA are presented developing perforation of the bowel at primary diagnosis after 7 respectively 10 days of initiation of high-dose glucocorticosteroides (GC) therapy.FindingsTwo male patients, aged 32 (case 1) and 36 years (case 2), were admitted to the hospital with the symptoms of dyspnea, fatigue, fever, and chest pain. The patients completed the previously published revised international Chapel Hill nomenclature of EGPA. The two patients (case 1 and 2) developed acute abdominal pain after 7 (case 1) and ten days (case 2) on GC treatment. Computed tomography followed by surgery detected acute perforation of the small intestine (middle part of jejunum, case 1) and colon transversum (case 2). The resected specimens disclosed bowel perforation with severe transmural inflammation, edema, hemorrhage and vasculitis typically of EGPA. On the first post-operative day, therapy with cyclophosphamide (according to the Austin protocol) with a dosage of 750 mg/qm every 3 weeks (for 8 cycles) was initiated with good response within three months of treatment.ConclusionThe course of disease of the two presented EGPA patients suggest that very early initiation of intensified immunosuppressive treatment, preferentially with cyclophophomide or B cell depletion strategies, needs to be considered to avoid life-threatening complications of GI involvement.

show abstract

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Gastrointestinal perforation due to vasculitis at primary diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA) despite a high dose glucocorticosteroids treatment

et al. 2014

View full text Add to dashboard Cite

show abstract

“…As a result, his abdominal signs dramatically subsided, leading to a signifi- Although gastrointestinal involvement is reported to be higher in children with CSS than that in adults with CSS (Zwerina et al 2008), long-lasting severe abdominal signs, as observed in our patient, have rarely been reported. Since massive use of corticosteroids increases the risk of intestinal perforation (Venditti et al 2011), we suggest that an aggressive immunosuppressive regimen including Tac is beneficial for children with refractory CSS like our case, although further studies are needed. Some previous reports have suggested a potentially causal relationship between treatment with leukotriene modifiers and the onset of CSS (Kobayashi et al 2003;Boyer et al 2006;Kanda et al 2010;Salerno et al 2010,).…”

Section: Discussionmentioning

confidence: 84%

Successful Multidrug Treatment of a Pediatric Patient with Severe Churg-Strauss Syndrome Refractory to Prednisolone

Watanabe

Aizawa-Yashiro

Tsuruga

et al. 2011

Tohoku J. Exp. Med.

View full text Add to dashboard Cite

“…The association between diverticular perforation and hypercortisolism is still controversial. However, there are several reports of oral steroid therapy leading to gastrointestinal perforation (4) (15) (16) (17) (18) (19) (20) (21) (22) (23) (24). Excessive levels of steroids may cause gastrointestinal perforation by thinning the bowel lymphoid tissue, thereby diminishing resistance to bacterial invasion, slowing down the turnover of intestinal mucosal cells, and decreasing fibroblastic reparative activity (25).…”

Section: Discussionmentioning

confidence: 99%

Cushing's disease presenting with gastrointestinal perforation: a case report

Hara¹,

Akutsu²,

Yamamoto³

et al. 2013

Endocrinology, Diabetes &Amp; Metabolism Case Reports

View full text Add to dashboard Cite

Gastrointestinal perforation is a complication associated with steroid therapy or hypercortisolism, but it is rarely observed in patients with Cushing's disease in clinical practice, and only one case has been reported as a presenting symptom. Herein, we report a rare case of Cushing's disease in which a patient presented with gastrointestinal perforation as a symptom. A 79-year-old man complained of discomfort in the lower abdomen for 6 months. Based on the endocrinological and gastroenterological examinations, he was diagnosed with Cushing's disease with a perforation of the descending colon. After consultation with a gastroenterological surgeon, it was decided that colonic perforation could be conservatively observed without any oral intake and treated with parenteral administration of antibiotics because of the mild systemic inflammation and lack of abdominal guarding. Despite the marked elevated levels of serum cortisol, oral medication was not an option because of colonic perforation. Therefore, the patient was submitted to endonasal adenomectomy to normalize the levels of serum cortisol. Subsequently, a colostomy was successfully performed. Despite its rarity, physicians should be aware that gastrointestinal perforation may be associated with hypercortisolism, especially in elderly patients, and immediate diagnosis and treatment of this life-threatening condition are essential. If a perforation can be conservatively observed, endonasal adenomectomy prior to laparotomy is an alternative treatment option for hypercortisolism.Learning points Thus far, only one case of gastrointestinal perforation as a presenting clinical symptom of Cushing's disease has been reported.Physicians should be aware that gastrointestinal perforation might be associated with hypercortisolism in elderly patients because elevated levels of serum cortisol may mask the clinical signs of perforation. Because of this masking effect, the diagnosis of the perforation also tends to be delayed.Although parenteral administration of etomidate is a standard treatment option for decreasing the elevated levels of serum cortisol, endonasal adenomectomy prior to laparotomy is an alternative treatment option if etomidate therapy is unavailable.

show abstract

Bowel perforations in a patient affected by Churg–Strauss syndrome under high-dose steroid treatment: will alternative drugs reduce risk of surgery?

Cited by 15 publications

References 8 publications

Gastrointestinal perforation due to vasculitis at primary diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA) despite a high dose glucocorticosteroids treatment

Gastrointestinal perforation due to vasculitis at primary diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA) despite a high dose glucocorticosteroids treatment

Successful Multidrug Treatment of a Pediatric Patient with Severe Churg-Strauss Syndrome Refractory to Prednisolone

Cushing's disease presenting with gastrointestinal perforation: a case report

Contact Info

Product

Resources

About