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Critical care 2007,11,213のレビューでは下記の鑑別診断リストが呈示されている。一方、NEJN 359;14,2008のClinical-problem-solvingでは、
Goodpasture症候群、SLE、Wegener肉芽腫症、顕微鏡的多発血管炎が挙げられている。
(症例の臨床症状によるが、混合型クリオグロブリン血症やTMA(血栓性微小血管障害)は鑑別診断にあげておいても良いと思われる)

Pulmonary –renal syndromes                
(1) Pulmonary renal syndrome associated with anti-GBM antibodies: Goodpasture’s syndrome
(2) Pulmonary renal syndrome in ANCA-positive systemic vasculitis  
 Wegener’s granulomatosis  
 Microscopic polyangiitis  
 Churg Strauss syndrome  
 Other vasculitis
(3) Pulmonary renal syndrome in ANCA-negative systemic vasculitis  
 Henoch Schönlein purpura  
 Mixed cryoglobulinaemia  
 Behçet’s disease  
 IgA nephropathy
(4) ANCA-positive pulmonary renal syndrome without systemic vasculitis:
  Idiopathic pulmonary renal syndrome  
 Pauci-immune necrotic glomerulonephritis and pulmonary capillaritis
(5) Pulmonary renal syndrome in drug-associated ANCA-positive vasculitis  
 Propylthiouracil  
 D-Penicillamine  
 Hydralazine  
 Allopurinol  
 Sulfasalazine
(6) Pulmonary renal syndrome in anti-GBM-postive and ANCA-positive patients
(7) Pulmonary renal syndrome in autoimmune rheumatic diseases (immune complexes and/or ANCA mediated)    
 Systemic lupus erythematosus  
 Scleroderma (ANCA?)  
 Polymyositis  
 Rheumatoid arthritis  
 Mixed collagen vascular disease
(8) Pulmonary renal syndrome in thrombotic microangiopathy  Antiphospholipid syndrome  
 Thrombotic thrombocytopenic purpura  
 Infections  
 Neoplasms
(9) Diffuse alveolar haemorrhage complicating idiopathic pauci-immune glomerulonephritis

追加 
Pulmonary-Renal Vasculitic Disorders:
Differential Diagnosis and Management
(Current Rheumatology Reports 2003, 5:107–115)

表 Pulmonary-renal
syndrome (pulmonary hemorrhage and
glomerulonephritis)の分類
(1) Goodpasture's syndrome or antiglomerular basement
membranes
(2) Immune complex–induced
 Systemic lupus erythematosus
 Rheumatoid arthritis
 Systemic sclerosis
(3) Antineutrophil cytoplasmic antibody–associated vasculitis
 Microscopic polyangiitis
 Wegener's granulomatosis
 Churg-Strauss syndrome
 Drug-induced vasculitis
 Pauci-immune necrotizing and crescentic
glomerulonephritis
(4) Non–antineutrophil cytoplasmic antibody–associated vasculitis
 Henoch-Schönlein purpura
 Essential cryoglobulinemia
 Behçet's disease
 Immunoglobulin A nephropathy
(5) Antiphospholipid syndrome
(6) Others

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