Pulmonary-Renal Syndrome, Diffuse PulmonaryHemorrhage and Glomerulonephritis, Associatedwith Wegener's Granulomatosis EffectivelyTreated with Early Plasma Exchange Therapy

Abstract

We present a case of a 38-year-old Japanese man with Wegener's granulomatosis complicated with pulmonary-renal syndrome, i.e., diffuse pulmonary hemorrhage and rapidly progressive renal glomerulonephritis. As this is a life-threatening condition, we promptly initiated plasma exchange with intravenous methylprednisolone therapy. Diffuse pulmonary hemorrhage and renal failure were markedly improved. This case merits presentation because there are few clinical studies of the treatment of Wegener's granulomatosis with pulmonary-renal syndrome, particularly with pulmonary hemorrhage.

Pulmonary Thromboembolism and Diffuse Alveolar Hemorrhage in Granulomatosis With Polyangiitis Vasculitis

“…9 In addition, plasma-exchange therapy may better preserve renal function, with less probability of developing end-stage renal disease. 10 For DAH, previous studies have demonstrated that a combination of remission-induction therapy and plasma-exchange therapy improves pulmonary infiltrates, respiratory function, and mortality rates, 11,12 suggesting that active pulmonary hemorrhage could be an indication of early plasmapheresis. However, there is no consensus in the use of plasma-exchange therapy in pulmonary hemorrhage.…”

Unusual Evolution in Wegener's Granulomatosis: Recovery of Pulmonary Involvement While Renal Disease Progressed to End-Stage

“…Other authors recommend PE therapy in diffuse alveolar hemorrhage 16 but EULAR recommendations for PE do not include extra-renal manifestations. 1 No study on PE in WG has focused on pulmonary involvement alone.…”

Treatment of renal manifestations of ANCA-associated Vasculitis

“…At least 50% of patients with ANCA-associated glomerulonephritis have pulmonary disease. Massive pulmonary hemorrhage affects about 10% of patients with ANCA glomerulonephritis, and is associated with an elevated risk of death (16). Constitutional signs and symptoms, such as fever, myalgias, arthralgias, and malaise, often accompany small-vessel vasculitis.…”

“…The duration of induction therapy is 3-6 months (figure 2). In the presence of kidney failure, plasma exchange (plasmapheresis) is often used in addition to pharmacological treatment (16,21,22). Once remission is achieved, treatment is scaled back to maintenance therapy with lower doses of GC, while cyclophosphamide is replaced by a less tox-ic immunosuppressant, such as azathioprine.…”

“…Patients presenting with pulmonary hemorrhage benefit also from the institution of plasmapheresis in a regimen similar to that used for patients with Goodpasture's disease or anti-GBM disease (16). Plasmapheresis is typically performed daily until the pulmonary hemorrhage ceases and then every other day for a total of seven to ten treatments.…”