A case of membranous nephropathy associated with Sjögren syndrome, polymyositis and autoimmune hepatitis.

Sjögren syndrome (SS) is a chronic systemic autoimmune disease characterized by lymphocytic infiltration of exocrine glands, especially lacrimal and salivary. The immunologic process which occurs in this syndrome is B cell hyperactivity, which results in production of autoantibodies and immune complexes. SS can exist as a primary disorder or in association with other autoimmune processes. A usually mild, proximal and insidious inflammatory myopathy can occur in patients with SS with a broad clinical and pathological spectrum. Interstitial nephritis with mild proteinuria and tubular dysfunction is the most common renal manifestation of SS, but glomerular involvement due to immune complex deposition may also rarely occur [Goules et al. 2000]. There is an association of SS with hepatic abnormalities, as evidenced by abnormal liver biochemical tests or histological characteristics of primary biliary cirrhosis (PBC), portal tract fibrosis, or autoimmune hepatitis [Abraham et al. 2004]. The pathogenetic mechanism of liver involvement in SS is not clear, but it is possible that hepatic and salivary gland damage share a similar pathology. The combination of Sjögren syndrome with kidney, liver and muscle involvement in one entity is extremely rare and data in the literature are remarkably sparse. We present a case of a 43-year-old female patient suffering from SS accompanied by polymyositis, membranous nephropathy and autoimmune hepatitis.

Acute renal failure after antibiotic-impregnated bone cement treatment of an infected total knee arthroplasty.

Antibiotic-impregnated cement is used frequently in revision procedures of infected total hip and knee arthroplasties. Local antibiotic treatment is as effective as the use of systemic antibiotics. The purpose of such treatment is to provide high tissue concentrations of antibiotics and minimize systemic toxicity, especially nephrotoxicity. Though antibiotic-impregnated cement is considered safe in terms of nephrotoxicity, two cases that have implicated aminoglycoside-impregnated cement in acute renal failure (ARF) after surgery for an infected total knee arthroplasty (TKA) have been reported [Curtis et al. 2005, Van Raaij et al. 2002]. Two more cases of postoperative ARF after use of combined tobramycin- plus vancomycin-impregnated cement, this time in total hip arthroplasty, have been recently reported [Patrick et al. 2006]. We report a case of ARF in a 61-year-old patient with a history of diabetes mellitus and hypertension after treatment of a febrile infection of a TKA with combined gentamicin- plus vancomycin-impregnated cement. The ARF could not sufficiently be attributed to other causes and though serum concentrations of antibiotics obtained from the 8th postoperative day and thereafter were far below the trough levels associated with nephrotoxicity, gentamicin and vancomycin seem to have contributed significantly to ARF in our case.

Pulmonary renal syndrome in an adult patient with Henoch-Shönlein purpura.

Henoch-Schönlein purpura (HSP) is a small vessel vasculitis characterized by purpuric skin rash, haematuria, abdominal pain, gastrointestinal bleeding and arthritis. Nephritis is more frequent and severe in adults than in children, with relatively more adults developing renal insufficiency. Another, fortunately rare, manifestation of HSP that increases mortality significantly, is diffuse alveolar haemorrhage. We report a rare case of an adult male patient with full-blown HSP that followed a respiratory tract infection. He successively, but not concurrently, developed all the clinical manifestations of HSP, i.e. arthritis, abdominal pain and bloody stools, a non-thrombocytopenic purpuric rash, and renal involvement; nephrotic range proteinuria first and haemodialysis-requiring nephritic syndrome later. Most interesting he developed life-threatening pulmonary haemorrhage fulfilling the criteria of the pulmonary-renal syndrome. An immunosuppressive regimen consisting of intravenous cyclophosphamide and corticosteroids was administered with success. In conclusion, HSP should be considered in the diagnosis of pulmonary-renal syndrome. In our opinion, the severity of the condition justifies the use of aggressive immunosuppressive treatment, like the one applied successfully to our patient.