Focal Segmental Glomerulosclerosis Associated with Primary Biliary Cirrhosis

To the Editor

Focal segmental glomerulosclerosis (FSGS) is a clinicopathological disease defined by the presence of proteinuria and segmental glomerular scars involving some glomeruli. Diagnosis is based on clinical and histopathological findings.¹ FSGS has primary and secondary etiologies, including drugs, viral infections, hemodynamic factors, malignancies, and postinflammatory processes.²

A 56-year-old female was admitted to our clinic with overt edema. After her physical examination and basic laboratory evaluation, kidney biopsy was performed to diagnose the etiology of nephrotic syndrome. Kidney biopsy revealed classical FSGS, so 1 mg/kg steroid, 5 mg/kg cyclosporin, and 20 mg/day atorvastatin therapy was initiated besides her previous medications which consisted of angiotensin receptor blocker (ARB) losartan and levothyroxine for primary hypertension and hypothyroidism, respectively. Partial remission was achieved in 4 weeks, her steroid therapy has been reduced to 5 mg/day because of proximal myopathy, and cyclosporine treatment was finalized due to development of diabetes mellitus and cataract. In her further visits the patient developed relapse and her 24 h urine protein level increased besides her transaminase levels and statin therapy was interrupted. Magnetic resonance cholangiopancreatography was performed due to the clinical suspicion of cholestasis. Antimitochondrial antibody M2 was detected as positive, and the liver biopsy revealed primary biliary cirrhosis (PBS) (Figure 1). After initiation of ursodeoxycholic acid treatment partial remission was achieved without any immunosuppressive agents.

Figure 1. Liver biopy showing primary biliary cirrhosis.

PBS is a slowly progressive autoimmune disease of the liver that primarily affects women. Progressive bile duct injury from portal and periportal inflammation could result in progressive fibrosis and eventually cirrhosis. Immunological and genetic factors might cause the disease. PBS often is associated with autoimmune diseases, including Sjogren’s syndrome, rheumatoid arthritis, and hypothyroidism.³ PBS is rarely associated with development of membranous nephropathy.⁴ It has been reported that glomerulopathies accompanying PBS may be effectively treated by using cyclosporine.⁵

It is important to recognize that FSGS is a nonspecific histologic finding, not a single disease. It can be observed in a variety of secondary settings with/without known causes besides primary idiopathic FSGS.

PBS and FSGS are chronic diseases and both these conditions are fairly rare. To date the likelihood of these uncommon conditions occurring in the same patient has not been reported. The disease itself or medications might induce or exacerbate an autoimmune response, leading to bile duct injury. In conclusion, we present a case of an unusual association between FSGS and PBS, and to our knowledge, this is the first case reported in the literature. During the course of FSGS, the clinicians kept in mind autoimmune liver diseases in patient with elevated liver enzymes.

Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.