Combination therapy with PSL and TAC appeared to be well tolerated and effective in suppressing the disease activity of SSc-PIP.

summaryA 51-year-old man was detected nasal bleeding, multiple pulmonary nodule and mass, urinalysis abnormality, renal involvement and high titer of proteinase 3-anti-neutrophil cytoplasmic antibody (PR3-ANCA), and was suspected of granulomatosis with polyangiitis and initiated with steroid pulse therapy. On the day after the start of steroid pulse therapy, generalized peritonitis due to ileal perforation occurred, and emergency ileectomy and peritonitis surgery were performed. Induction therapy with steroid pulse therapy, plasma exchange and intravenous cyclophosphamide therapy (IVCY) and maintenance therapy with glucocorticoid and azathioprine led to good therapeutic outcomes. Gastrointestinal perforation in GPA is a rare complication, and we examined the clinical features, treatment contents, and prognosis of GPA with gastrointestinal perforation from this case and previous reports. Lung involvements were complicated in all reported cases. Gastrointestinal perforations in GPA were frequent in the small intestine, occurred just before and immediately after the start of treatment, and were severe involvement with poor prognosis because of the high mortality rate (46.7%). The frequency of ear, nose and upper respiratory tract lesions in the surviving group was significantly higher than in the dead group (survival 87.5%, death 28.3%, P = 0.041). IVCY were more frequently used in the surviving group (62.5%) than the death group (16.7%), but it was not significantly. GPA complicated with gastrointestinal perforation is a severe condition with poor prognosis, but there is a possibility to improve prognosis by early diagnosis and early initiation of strong treatment.

Objectives We retrospectively compared the therapeutic effects of combination therapy with prednisolone (PSL) and oral tacrolimus (TAC) or azathioprine (AZA) on progressive interstitial pneumonia with systemic sclerosis (SSc-PIP). Methods The effects of PSL (0.2–0.5 mg/kg/day) and TAC (3 mg/day) or AZA (1–2 mg/kg/day) therapies (n = 18) were evaluated for short (12 months) and long (36 months or more) periods. Results In the short period, IP improved in 6 and 5 patients and was stable in 12 and 13 patients in the TAC and AZA groups, respectively. In the long period, 11 patients were followed up in the TAC group and 12 in the AZA group. IP improved in 4 and 2 patients and was stable in seven and nine in the TAC and AZA groups, respectively. The rates of evolution of total fibrosis score, and those corrected by disease duration for the long period, in the TAC group were significantly lower than those in the AZA group (p = .017 and .025, respectively). Conclusion The inhibitory effect of PSL and TAC combination therapy on the progression of fibrosis in SSc-PIP may be superior to that of PSL and AZA in the long period.

Although left ventricular (LV) systolic dysfunction in patients suffering from Takayasu arteritis (TA) has been reported, little is known regarding the development of heart failure in these patients. We report a novel finding of active TA and familial hypercholesterolaemia presenting with severe LV dysfunction through multimodality assessments of LV systolic dysfunction.

These findings suggest that the levels of KL-6 before and after treatment in ARS-DMIP may represent the disease activity of IP, and they may be useful as the predictor of relapse in IP in patients with ARS-DMIP.