ABSTRACT
BACKGROUND: Eosinophilic 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. FINDINGS: Two 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. CONCLUSION: The 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.
ABSTRACT
OBJECTIVE: Painful swelling of the anterior chest wall caused by osteitis and hyperostosis in the sternocostoclavicular region are characteristically observed in patients suffering from SAPHO syndrome. Autoimmune triggering of inflammation and bacterial infection is hypothesized to be involved in the pathogenesis. Promising treatment modalities include antirheumatic and antibiotic medications. METHODS: Ten patients with SAPHO syndrome and symptomatic osteitis in the sternocostoclavicular region were treated by a single instillation of glucocorticosteroids (20 mg triamcinolone) into the sternocostoclavicular joints. The disease activity was evaluated on the basis of a questionnaire asking for osteitis activity (quantified for complains on a scale of 0-6), by Health Assessment Questionnaire (HAQ) score, erythrocyte sedimentation rate, C-reactive protein, and magnet resonance imaging (MRI) scanning of the sternocostoclavicular region (osteitis scores quantified for inflammation on a scale of 0-2 by the radiologist) prior to injection and after 12 weeks. No changes of the preexisting antirheumatic therapy were allowed during the observation interval. RESULTS: All patients continued the study during the follow-up. The osteitis score changed from 4.2 (mean; standard error (SE) ±0.3) to 3.2 (±0.4), [P = 0.062], the erythrocyte sedimentation rate from 19.0 (range from 12 to 30) to 19.9 (from 12 to 27), [P = 0.430], and the MRI score from 1.6 (±0.2) to 1.5 (±0.2) [P = 1.0]. One patient developed an increase of the clinical osteitis activity from 3 to 5 according the scoring system; only 2 patients showed a reduction of the MRI activity score from 2 to 1. CONCLUSIONS: Intra-articular glucocorticosteroid instillation does not appear to reduce osteitis in the sternocostoclavicular region in patients with SAPHO syndrome.
