Early and late humoral rejection: a clinicopathologic entity in two times.

Humoral rejection is an important cause of early and late graft loss. The late variant is difficult to diagnose and treat. There is a close correlation between sclerosing nephropathy and anti-HLA antibodies. We analyzed 113 renal allograft recipients between August 2004 and April 2007. Acute humoral rejection was defined as acute graft dysfunction in presence of donor-specific antibodies (DSA) detected by flow panel reactive antibodies (PRA) and/or C4d positive pericapilary tubules (PTC) detected histopathologically by immunofluorescent or immunoperoxidase at less than 3 months postransplantation. Late humoral rejection was defined as dysfunction occurring after 3 months postransplantation with histopathologic glomerulopathy or vasculopathy and positive C4d PTC. We included all patients who were diagnosed with early or late graft dysfunction and underwent biopsy, all of which were examined for C4d. Four patients had acute humoral rejection treated with IVIG or plasmapheresis. The patient and graft survivals were 100% and serum creatinine averaged 1.7 mg/dL. Three recipients experienced late humoral rejection at 3 to 10 years posttransplantation All received high-dose IVIG; one also was treated with thymoglobulin. Immunosuppression was switched to tacrolimus, mycophenolate mofetil, and steroids. Only one patient recovered renal function; the others returned to dialysis. Among seven patients only one had an actual PRA (>20%) and three showed 10% to 20%. However, six had a positive historical PRA of 10% to 50%. In conclusion, Recognition of acute humoral rejection has contributed to graft rescue by controlling alloantibody production through new specific immunosuppressive therapies in contrast with the clinical response to acute therapy, treatment of a chronic entity has shown poor outcomes, probably because antibody mediated chronic graft damage is already present when the late diagnosis is established by biopsy.

[Nail-patella syndrome associated with monoclonal gammopathy. Review regarding a case]. / Síndrome uña-rótula asociado a gammopatia monoclonal. Revisión a propósito de un caso.

A 54 years old female with significant impairment of renal function is presented. On physical examination, the presence of dystrophic nails, elbow dysplasia and prominent iliac horns. Familial study showed similar nail and bone deformities in 3 of six sons. The nail patella syndrome diagnosis was based on these findings. This is a rare autosomal dominant hereditary disease, probably related to congenital alterations in collagen metabolism. Clinical characteristics include bone abnormalities that principally involve knees and elbows, nail alterations and the presence of iliac horns, that are considered pathognomonic of the syndrome. Renal involvement is observed in 30 to 55% of cases. This patient had also an IgA-lambda paraprotein, whose relationship to the above mentioned syndrome is uncertain, since no evidences of malignant plasma cell dyscrasia were demonstrated.