ORIGINAL ARTICLE

Pulmonary-renal syndrome in systemic sclerosis: a report of three cases and review of the literature

Authors

Taio Naniwa¹, Shogo Banno¹, Yoshiki Sugiura², Kaori Yokota³, Tomoyo Oosawa¹, Shinji Maeda¹, Yoshihito Hayami¹, Nobuyuki Takahashi², Ryuzo Ueda¹ and Yoshifuji Matsumoto⁴

1. Division of Rheumatology, Nagoya City University Hospital, Department of Internal Medicine and Molecular Sciences, Nagoya City University Graduate School of Medical Sciences, Kawasumi, Mizuho-ku, Nagoya 467-8601, Japan
2. Department of Internal Medicine 4, Nagoya Higashi Municipal Hospital, Nagoya, Japan
3. Department of Rheumatology, Gifu Prefectural Tajimi Hospital, Gifu, Japan
4. Division of Internal Medicine, Nanakuri Sanatrium, Fujita Health University, Mie, Japan

Received:

09 August 2006

Accepted:

18 October 2006

Full Text

Download (PDF)

Abstract

We describe three cases of acute renal failure with diffuse alveolar hemorrhage, which is designated pulmonary-renal syndrome (PRS), in systemic sclerosis (SSc) and review the literature to better define this rare but severe complication of SSc. The clinical course of three SSc patients with acute renal failure and concomitant diffuse alveolar hemorrhage are reported, and the literature published between 1967 and 2005 is reviewed following a PubMed search. Including our cases, a total of 19 SSc patients with acute renal failure and concomitant diffuse alveolar hemorrhage have been reported. Pulmonary-renal syndrome developing in SSc patients can be categorized clinicopathologically into three entities: PRS with thrombotic microangiopathy, PRS with small vessel vasculitides accompanied with SSc, and d-penicillamine-induced Goodpasture-like syndrome. Patients with scleroderma PRS with thrombotic microangiopathy, to which group our all patients belong, often developed diffuse alveolar hemorrhage after receiving high-dose corticosteroid therapy. Pulmonary-renal syndrome is a fatal complication of SSc and results from different pathogenic processes. Prompt differential diagnosis between the subsets is critical, because therapeutic strategy may differ in the use of high-dose corticosteroid and plasma exchange between the subsets of PRS. Clinical courses of the patients with PRS with thrombotic microangiopathy suggest that high-dose corticosteroid therapy is a trigger of diffuse alveolar hemorrhage in patients with diffuse SSc with signs of thrombotic microangiopathy.

Key words

Acute renal failure - Diffuse alveolar hemorrhage - Scleroderma - Scleroderma renal crisis - Thrombotic microangiopathy