Figures
- 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
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
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
- TABLE 1
Differential diagnoses of pulmonary renal syndrome
ANCA-associated vasculitis Granulomatosis with polyangiitis (GPA) Microscopic polyangiitis (MPA) Eosinophilic granulomatosis with polyangiitis (EGPA) Anti-GBM disease Anti-GBM disease ANCA-negative vasculitis IgA disease Cryoglobulinaemia Drug-induced vasculitis Cocaine d-penicillamine Autoimmune connective tissue disease Systemic 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 disease Anti-GBM antibodies are 95–100% sensitive and 90–100% specific [10, 63, 64] ANCA-negative vasculitis IgA disease Nil specific Cryoglobulinaemia Hepatitis 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 dermatomyositis Anti-Jo1, anti-Ro antibodies Systemic sclerosis Anti-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.
