Recovery of renal function in undifferentiated connective tissue disease after treatment with angiotensin-converting enzyme inhibitors

Angiotensin-converting enzyme (ACE) inhibitor therapy has been reported to improve patient survival and promote recovery of renal function in the renal crisis of systemic sclerosis. In addition, an ACE inhibitor and a calcium channel blocker have been reported to control hypertension and reverse dialysis-dependent renal failure in a patient with undifferentiated connective tissue disease. We treated a patient with undifferentiated connective tissue disease who developed hypertension, pulmonary compromise, and renal failure requiring prolonged dialysis therapy. Due to allergy, the patient's hypertension could not be treated with ACE inhibitors initially, yet pulmonary function improved and renal function partially recovered with tenormin and minipress. When blood pressure became refractory to tenormin and minipress after 14 months of peritoneal dialysis, the patient was treated with lisinopril alone. Pulmonary function has remained stable and the patient has been off renal replacement therapy for 26 months, with a further substantial increase in creatinine clearance following treatment with lisinopril. The delayed and sustained recovery of renal and pulmonary function in the present case suggests undifferentiated connective tissue disease, like systemic sclerosis, may benefit from therapy with ACE inhibitors.     

Glomerulonephritis in children with mixed connective tissue disease

AIMS: Mixed connective tissue disease (MCTD) has overlapping clinical features with systemic lupus erythematosus (SLE). Renal biopsy is necessary for all children with SLE to evaluate the prognosis, because they are at a quite high risk of developing renal complications. Furthermore, lupus nephritis and hypocomplementemia usually precede the appearance of clinical manifestations. Immune complex-mediated nephritis is one of the major complications of MCTD. Juvenile MCTD is known to be associated with a higher risk of nephritis than adult MCTD. However, it is uncertain whether all children with MCTD should be subjected to a renal biopsy, and whether most of those with hypocomplementemia present nephropathy, as in patients with SLE. We examined the histopathological characteristics of juvenile MCTD nephritis, the importance of renal biopsy and the implications of hypocomplementemia in our patients and reported cases of MCTD. MATERIAL AND METHODS: We performed renal biopsy in 11 children with MCTD and found 6 patients with glomerulonephritis. In addition, we studied the frequency and the characteristics of glomerulonephritis in 71 cases of juvenile MCTD (our 11 patients and 60 reported cases). We also analyzed the relationship between hypocomplementemia and pathological features in 41 cases of MCTD nephritis (23 adults, 18 children). RESULTS: 6 of our 11 patients had glomerulonephritis, but of them four had no abnormality in urinalysis at the time of biopsy. In 5 patients renal biopsy showed normal findings. Review of 71 cases of juvenile MCTD showed that of them 28% presented latent asymptomatic nephritis at the time of biopsy. Membranous nephropathy (MN) and mesangial proliferative glomerulonephritis (MPG) were common in MCTD. Interestingly, hypocomplementemia was more frequently observed in patients with MN or mixed form of MN and MPG (MPG/MN) than simple MPG based on our review of 41 cases (p < 0.01). CONCLUSION: A more aggressive indication of renal biopsy should be considered in children with MCTD because of the high incidence of non-clinical nephritis. The hypocomplementemia observed in patients with MCTD suggests the high frequency of glomerulonephritis, including membranous lesions.     

The hand in mixed connective tissue disease.

The mixed connective tissue disease syndrome has been described in the medical literature. The clinical and serological characteristics of the syndrome are defined in this paper. The hands of these patients differ from the hands of patients with systemic lupus, rheumatoid arthritis, or systemic sclerosis. In 10 patients there were no erosive changes on radiological examination and all 10 patients had Raynaud's phenomenon. The most striking finding was tightness in the flexors. Mild cases of flexor tightness improved with systemic steroids. One patient with severe flexor tightness required surgical release of adhesions from a chronic inflammatory process of fascia, muscle, and tenosynovium. Biochemical studies showed an abnormal collagen pattern that may be distinct for mixed connective tissue disease.     

A case of mixed connective tissue disease (MCTD) complicated with MPO-ANCA-related necrotizing glomerulonephritis

Renal diseases of mixed connective tissue disease (MCTD) are not unusual. Although most of them are SLE-like renal impairment with immune complex deposits, systemic sclerosis- (SSc) like renal impairments with intimal thickening of interlobular arteries or arterioles are also encountered. Several cases of SSc complicated with MPO-ANCA-related necrotizing glomerulonephritis (nGN) are reported. Here we report a case which developed MPO-ANCA-related nGN 16 years after the diagnosis of MCTD. She exhibited pauci-immune focal nGN and significantly high titer of MPO-ANCA. She was successfully treated with prednisolone and cyclophosphamide. We believe this is the first case in which MPO-ANCA-related nGN was demonstrated in a patient with MCTD.     

Collapsing glomerulopathy in a patient with mixed connective tissue disease

Collapsing glomerulopathy (CG) is a distinct clinicopathological entity characterized by glomerular capillary collapse, podocyte proliferation, diffuse mesangial sclerosis, and podocyte maturation arrest. Initially noted primarily in HIV infected patients, a number of other diseases have now been associated with CG. Mixed connective tissue disease (MCTD) is a disease with overlapping features of systemic lupus erythematosus, progressive systemic sclerosis, and polymyositis. It was originally thought that renal involvement was a rare complication of MCTD. However, over the years, it has become clearer that renal involvement, although not always clinically apparent, is frequent. In this report we present a patient with MCTD who developed CG.     

混合性结缔组织病肾损害四例

1972年Sharp等将一组具有多种结缔组织病临床表现，且血清学上表现为高滴度斑点型抗核抗体（ANA）和抗核糖核蛋白抗体（RNP）抗体的结缔组织病，称为混合性结缔组织病（MCTD）。Kitridou等认为MCTD的肾损害发生率为10％-50％：而国内相关的报道少见。我们分析了本院4例MCTD肾损害的临床病理表现，以进一步提高对此类疾病的认识。     

A case of pneumatosis cystoides intestinalis in connective tissue disease

Pneumatosis cystoides intestinalis (PCI) with spontaneous pneumoperitoneum is a rare but important condition in patients with connective tissue disease. In the present study, we report on a woman with mixed connective tissue disease who presented with PCI, and subsequently symptoms, of chronic intestinal pseudo‐obstruction. Prompt surgical resection of the bowel segment with pneumatosis should be considered to prevent a condition of gas leakage from turning into full‐blown chemical peritonitis.     

Pregnancy outcome in nephrotic syndrome with mixed connective tissue disease

We describe two pregnancies of a young woman with mixed connective tissue disease. In June 1983, she was diagnosed as having Raynaud's phenomenon, arthralgia, and proteinuria. She then developed nephrotic syndrome. Methylprednisolone was initially prescribed at a large dose of 1 g/day which was slowly tapered to 5 mg/day. The proteinuria disappeared. During both pregnancies (the first beginning in December 1988 and the second in May 1992), the patient was placed on a prednisolone maintenance dose (5 mg/day). Both neonates were born healthy at term with no complications. Continuing prednisolone may be useful in pregnant women, and aggressive treatment to prevent mixed connective tissue disease exacerbation may be appropriate during pregnancy.     

Panniculitis in a patient on methotrexate for mixed connective tissue disease

Accelerated nodulosis during methotrexate therapy for rheumatoid arthritis has been well described. There have been recent reports of nodulosis in patients on methotrexate for other inflammatory conditions. Panniculitis is a newly discovered pathological entity in this setting. We describe a case of panniculitis in a woman receiving methotrexate for mixed connective tissue disease.     

A morbid course in a girl with mixed connective tissue disease

 We describe an 18-year-old girl with a 13-year history of mixed connective tissue disease whose clinical course was unique: she ultimately developed end-stage kidney and bowel disease that led to a protracted morbid clinical course. We report this case to alert pediatric nephrologists to the importance of early recognition of possible intestinal disease in these patients. Unfortunately, no therapy is currently known to reverse the pathological process in the bowel, but multiorgan transplantation might be an option if the numerous medical complications of end-stage bowel disease can be successfully controlled. Received: 7 July 1997 / Revised: 4 December 1997 / Accepted: 29 May 1998