Pulmonary renal syndrome associated with Wegener's granulomatosis: a case report and review of literature

Clin Exp Nephrol (2010) 14:8084

DOI 10.1007/s10157-009-0223-1

CASE REPORT

Pulmonary renal syndrome associated with Wegeners granulomatosis: a case report and review of literature

Ali A. Al bshabshe Hussein Al-Khalidy Hyder A. Omer Daefullah H. Al-Amri Aymen Hamad Saad-Rezk Abdulwahed Mahmoud R. Hussein

Received: 14 March 2009 / Accepted: 13 August 2009 / Published online: 4 September 2009 Japanese Society of Nephrology 2009

Abstract Pulmonary renal syndromes are rare but serious complications of systemic vasculitis. The majority of these cases are related to ANCA-associated vasculitis. These syndromes represent a combination of diffuse alveolar hemorrhage and rapidly progressive glomerulonephritis. Here we report a case of 21-year-old female with a clinical picture of community-acquired pneumonia that rapidly evolved to pulmonary hemorrhage and acute renal failure. Combined pulse steroid treatment, immunosuppressive treatment, and plasmapheresis were instituted and resulted in the improvement of the patient. The diagnosis of Wegeners granulomatosis was established based on the clinical and serological ndings (positive cANCA). Here we review the relevant literature on pulmonary renal syndromes associated with Wegeners granulomatosis and discuss their diverse clinicopathologic features.

Keywords Wegeners granulomatosis Plasmapheresis

Pulmonary renal syndrome

Introduction

Wegeners granulomatosis was rst recognized as a distinct clinicopathologic entity in 1936, when Wegener reported three patients with a peculiar rhinogenic granulomatosis. Goodman and Churg summarized several postmortem studies in 1954 from which the classic triad of this condition evolved. The clinicopathologic complex of Wegeners granulomatosis is characterized by necrotizing and granulomatous inammation of the upper and lower respiratory tracts, glomerulonephritis, and systemic vasculitis. Multiple organs are often involved, but there is a predilection for the upper and lower respiratory tracts, the eyes, and the kidneys [1].

With the recognition of an association between antineutrophil cytoplasmic antibodies (ANCAs) and Wegeners granulomatosis, the concept of Wegeners granulomatosis has been modied and the need to demonstrate granulomatous inammation as a prerequisite for the diagnosis of Wegeners granulomatosis has been challenged [2]. Therefore, a less restrictive denition has been proposed, termed Wegeners vasculitis. This less restrictive category includes ANCA-positive patients with clinical presentations of Wegeners granulomatosis, such as sinusitis, pulmonary inltrates, nephritis, and documented necrotizing vasculitis, but without biopsy-proven granulomatous inammation. Both classic Wegeners granulomatosis and Wegeners vasculitis are considered different manifestations of the same disease process. The term Wegeners syndrome is a more...