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Review
. 2022 Dec;18(4):220208.
doi: 10.1183/20734735.0208-2022. Epub 2023 Jan 10.

Pulmonary renal syndrome: a clinical review

Niamh Boyle  1 , Marissa O'Callaghan  1   2 , Ali Ataya  3 , Nishant Gupta  4 , Michael P Keane  1   2 , David J Murphy  2   5 , Cormac McCarthy  1   2
Affiliations
Review

Pulmonary renal syndrome: a clinical review

Niamh Boyle et al. Breathe (Sheff). 2022 Dec.

Abstract

The term "pulmonary renal syndrome" describes a clinical syndrome which is characterised by the presence of both diffuse alveolar haemorrhage and glomerulonephritis. It encompasses a group of diseases with distinctive clinical and radiological manifestations, as well as different pathophysiological processes. The most common diseases implicated are anti-neutrophil cytoplasm antibodies (ANCA)-positive small vessel vasculitis and anti-glomerular basement membrane (anti-GBM) disease. Prompt recognition is required as respiratory failure and end-stage renal failure can rapidly occur. Treatment includes a combination of glucocorticoids, immunosuppression, plasmapheresis and supportive measures. The use of targeted treatments has significantly reduced mortality. Thus, an understanding of pulmonary renal syndrome is essential for the respiratory physician.

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Conflict of interest statement

Conflict of interest: M.P. Keane has received consulting fees from Boehringer and Roche, outside the submitted work; payment or honoraria for lectures, presentations, speakers’ bureaus, manuscript writing or educational events from Boehringer, outside the submitted work; and support for attending meets and/or travel from Boehringer, AstraZeneca and Roche, outside the submitted work. C. McCarthy has received grants and speaker fees from Boehringer Ingelheim, and speaker fees from Roche Ltd. He is on the scientific advisory boards of the LAM Foundation and the European Pulmonary Fibrosis Federation. He has a consulting contract with Savara Pharmaceuticals as part of their clinical advisory board. C. McCarthy is a current member of the Breathe editorial board. The remaining authors have nothing to disclose.

Figures

FIGURE 1
FIGURE 1
High-resolution computed tomography (CT) images of the chest from patients with diffuse alveolar haemorrhage (DAH) in pulmonary renal syndrome. a–c) The axial CT slices show extensive bilateral mixed consolidative and ground-glass opacities with a mid to lower zone predominance admixed with coarsened interlobular septa, appearances typical for DAH. d) Milder disease is shown on an axial CT slice with patchy ground-glass opacities bilaterally. e) A coronal CT chest image highlights the mid to lower zone predominance in DAH.
FIGURE 2
FIGURE 2
Bronchoalveolar lavage (BAL) fluid specimens from a patient with diffuse alveolar haemorrhage (DAH) in pulmonary renal syndrome. Progressive haemorrhagic BAL can be noted in the serial samples (number 1 is the first BAL, number 2 is the second BAL, and number 3 is the third BAL).
FIGURE 3
FIGURE 3
Treatment algorithm in pulmonary renal syndrome. Treatment depends on the underlying cause; however, it is often a combination of glucocorticoid and immunosuppressant. Plasmapheresis can be considered in certain groups. Treatment typically consists of an induction phase followed by a maintenance phase. DAH: diffuse alveolar haemorrhage; AAV: ANCA-associated vasculitis; ANCA: anti-neutrophil cytoplasm antibodies; GPA: granulomatosis with polyangiitis; MPA: microscopic polyangiitis; EGPA: eosinophilic granulomatosis with polyangiitis; GBM: glomerular basement membrane; APS: antiphospholipid syndrome; SLE: systemic lupus erythematosus; CYC: cyclophosphamide; PLEX: plasmapheresis; IVIG: intravenous immunoglobulin; MMF: mycophenolate mofetil; AZA: azathioprine; MTX: methotrexate.
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Comment in

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