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

Abstract

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.

Rheumatoid arthritis presenting as rheumatoid meningitis: A case report

“…[2] Steroids are the mainstay of treatment, often supplemented with adjuvant cyclophosphamide or azathioprine. [2,3,10] The response to steroids can be rapid, with improvement of symptoms in weeks, as seen in our patient. [10] TNF blockers and MTX do not appear to be preventative, and rheumatoid meningitis has been documented to recur when switching from cyclophosphamide to infliximab.…”

“…[2,3,10] The response to steroids can be rapid, with improvement of symptoms in weeks, as seen in our patient. [10] TNF blockers and MTX do not appear to be preventative, and rheumatoid meningitis has been documented to recur when switching from cyclophosphamide to infliximab. [11,12] However, MTX and TNF blockers can be used as a steroid sparing agents for patients who wish to avoid other immunosuppressants such as cyclophosphamide or azathioprine, all of which have previously been used for treatment.…”

Successful Use of Plasma Exchange in the Treatment of Corticosteroid-Refractory Eosinophilic Granulomatosis with Polyangiitis Associated with Gastrointestinal Manifestations

“…EGPA is typically corticosteroid responsive with prompt treatment [ 7 ]. However, some patients develop severe complications, such as bowel perforation, infarction, or obstruction [ 7 ].…”

Recalcitrant steroid-resistant EGPA with multisystem involvement

“…[8,9] The presence of disabling myalgia at the time of hospitalization was also notable, as myalgia rarely represents a main presenting feature of EGPA and is most commonly seen during the prodromal phase of the illness. [4] Gastrointestinal involvement in EGPA is thought to be eosinophil-mediated, [10,11] which was not readily apparent on initial histopathologic evaluation of our patient's intestinal biopsy specimens. Staining with a monoclonal antibody targeting EPX however revealed intense staining in biopsy specimens taken from multiple sites in the gastrointestinal tract, providing evidence of prior eosinophil degranulation and pathophysiologic insight into our patient's unremitting abdominal pain.…”