Idiopathic pulmonary-renal syndrome

The clinical challenge of an underestimated condition

Abstract

The specific pulmonary-renal syndrome term (PRS) denotes simultaneous and continuously pulmonary hemorrhage (PH) and glomerulonephritis (GN). Therefore, the specific PRS implies a much more restrictive range of etiological possibilities and the most frequent is the small vessel vasculitides (SVVsVPV). As a consequence, it is known at present that the coexistence of the two affections, it means GN and PH, results from a series of illnesses with different underlying pathogenic mechanisms. The diagnosis of PRS is established by a combination of clinical presentation, serologic results and histological findings evaluated in an adequate clinical context. In the majority of patients with PH and GN, it is necessary to make a renal biopsy and final diagnostic test in the majority of cases; and with great value for the prognosis and treatment.

The objectives of the treatment in PRS include: To remove the circulating antibodies, to stop the autoantibodies production; and to remove any antigen that stimulates the antibodies production. We reported six PRS cases by vasculitic disorder in adult patients.