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Pulmonary renal syndrome: a clinical review

Niamh Boyle, Marissa O'Callaghan, Ali Ataya, Nishant Gupta, Michael P. Keane, David J. Murphy, Cormac McCarthy
Breathe 2022 18: 220208; DOI: 10.1183/20734735.0208-2022
Niamh Boyle
1Department of Respiratory Medicine, St. Vincent's University Hospital, Dublin, Ireland
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Marissa O'Callaghan
1Department of Respiratory Medicine, St. Vincent's University Hospital, Dublin, Ireland
2School of Medicine, University College Dublin, Dublin, Ireland
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Ali Ataya
3Division of Pulmonary, Critical Care and Sleep Medicine, University of Florida, Gainesville, FL, USA
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Nishant Gupta
4Division of Pulmonary, Critical Care and Sleep Medicine, University of Cincinnati, Cincinnati, OH, USA
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Michael P. Keane
1Department of Respiratory Medicine, St. Vincent's University Hospital, Dublin, Ireland
2School of Medicine, University College Dublin, Dublin, Ireland
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  • ORCID record for Michael P. Keane
David J. Murphy
2School of Medicine, University College Dublin, Dublin, Ireland
5Department of Radiology, St. Vincent's University Hospital, Dublin, Ireland
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Cormac McCarthy
1Department of Respiratory Medicine, St. Vincent's University Hospital, Dublin, Ireland
2School of Medicine, University College Dublin, Dublin, Ireland
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  • For correspondence: cormac.mccarthy@ucd.ie
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  • FIGURE 1
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    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
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    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
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    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.

Tables

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  • TABLE 1

    Differential diagnoses of pulmonary renal syndrome

    ANCA-associated vasculitisGranulomatosis with polyangiitis (GPA)
    Microscopic polyangiitis (MPA)
    Eosinophilic granulomatosis with polyangiitis (EGPA)
    Anti-GBM diseaseAnti-GBM disease
    ANCA-negative vasculitisIgA disease
    Cryoglobulinaemia
    Drug-induced vasculitisCocaine
    d-penicillamine
    Autoimmune connective tissue diseaseSystemic lupus erythematosus (SLE)
    Polymyositis
    Diabetes mellitus
    Mixed connective tissue disease (MCTD)
    Systemic sclerosis

    Classifying the differential diagnoses of pulmonary renal syndromes into groups based on the underlying pathological process. ANCA: anti-neutrophil cytoplasm antibodies; GBM: glomerular basement membrane.

    • TABLE 2

      Serology testing in pulmonary renal syndromes

      ANCA-associated vasculitis
       Granulomatosis with polyangiitis (GPA)ANCA positive 90% [61]; PR3 positive in 75%
       Microscopic polyangiitis (MPA)ANCA positive 60% [61]; MPO positive in 65%
       Eosinophilic granulomatosis with polyangiitis (EGPA)ANCA positive 30–70% [61]; PR3 positive in 5%, MPO positive in 45% [62]
      Anti-GBM diseaseAnti-GBM antibodies are 95–100% sensitive and 90–100% specific [10, 63, 64]
      ANCA-negative vasculitis
       IgA diseaseNil specific
       CryoglobulinaemiaHepatitis serology; serum cryoglobulins [65]
      Autoimmune connective tissue disease
       Systemic lupus erythematosus (SLE)Anti-dsDNA, anti-Smith, anti-C1q antibodies
       Antiphospholipid syndrome (APS)Anti-cardiolipin, lupus anticoagulant antibodies
       Rheumatoid arthritis (RA)Rheumatoid factor, anti-cyclic citrullinated peptides
      Mixed connective tissue disease (MCTD)Anti-RNP
       Polymyositis and dermatomyositisAnti-Jo1, anti-Ro antibodies
       Systemic sclerosisAnti-centromere, anti-Scl70 [60, 66]

      Positive serology can aid in narrowing the diagnosis in pulmonary renal syndrome. Differentiating between ANCA-positive and ANCA-negative serology is often the first step in determining the cause. ANCA: anti-neutrophil cytoplasm antibodies; GBM: glomerular basement membrane; PR3: proteinase-3; MPO: myeloperoxidase; dsDNA: double-stranded DNA.

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      Pulmonary renal syndrome: a clinical review
      Niamh Boyle, Marissa O'Callaghan, Ali Ataya, Nishant Gupta, Michael P. Keane, David J. Murphy, Cormac McCarthy
      Breathe Dec 2022, 18 (4) 220208; DOI: 10.1183/20734735.0208-2022

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      Pulmonary renal syndrome: a clinical review
      Niamh Boyle, Marissa O'Callaghan, Ali Ataya, Nishant Gupta, Michael P. Keane, David J. Murphy, Cormac McCarthy
      Breathe Dec 2022, 18 (4) 220208; DOI: 10.1183/20734735.0208-2022
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