Diagnosis of Pulmonary-Renal Syndrome - Flowchart
Diagnosis of Pulmonary-Renal Syndrome - Flowchart Pulmonary Renal Syndrome Pulmonary Renal Syndrome
«Flowchart»

Clinical features of PRS
(eg, constitutional prodrome + features of DAH RPGN)

Clinical features of PRS
(eg, constitutional prodrome + features of DAH RPGN)

Clinical features of PRS
(eg, constitutional prodrome + features of DAH RPGN)

Clinical features of PRS

“Double-positive” disease

“Double-positive” disease

“Double-positive” disease

“Double-positive” disease

Anti-GBM disease

Anti-GBM disease

Anti-GBM disease

Anti-GBM disease

ANCA-associated vasculitis

ANCA-associated vasculitis

ANCA-associated vasculitis

ANCA-associated vasculitis

Testing consistent with PRS

Testing consistent with PRS

Testing consistent with PRS

Testing consistent with PRS

ANCA+

ANCA+

ANCA+ ANCA+

Anti-GBM Ab+

Anti-GBM Ab+

Anti-GBM Ab+ Anti-GBM Ab+

ANCA+ and anti-GBM Ab+

ANCA+ and anti-GBM Ab+

ANCA+ and anti-GBM Ab+ ANCA+ and anti-GBM Ab+

ANCA and anti-GBM negative

ANCA and anti-GBM negative

ANCA and anti-GBM negative ANCA and anti-GBM negative

INDUCTION*

Glucocorticoids
Rituximab or cyclophosphamide +/– PLEX (if severe)

INDUCTION*

Glucocorticoids
Rituximab or cyclophosphamide +/– PLEX (if severe)

INDUCTION*

INDUCTION* *

Glucocorticoids
Rituximab or cyclophosphamide +/– PLEX (if severe)


MAINTENANCE*

Rituximab
Azathioprine or methotrexate or mycophenolate mofetil

MAINTENANCE*

Rituximab
Azathioprine or methotrexate or mycophenolate mofetil

MAINTENANCE*

MAINTENANCE* *

Rituximab
Azathioprine or methotrexate or mycophenolate mofetil


End

End

End

INDUCTION

Glucocorticoids
Cyclophosphamide PLEX

INDUCTION

Glucocorticoids
Cyclophosphamide PLEX

INDUCTION

INDUCTION

Glucocorticoids
Cyclophosphamide PLEX


MAINTENANCE

Not typically required

MAINTENANCE

Not typically required

MAINTENANCE

MAINTENANCE

Not typically required

INDUCTION

Glucocorticoids
Cyclophosphamide PLEX

INDUCTION

Glucocorticoids
Cyclophosphamide PLEX

INDUCTION

INDUCTION

Glucocorticoids
Cyclophosphamide PLEX


MAINTENANCE

Rituximab or Azathioprine or Methotrexate

MAINTENANCE

Rituximab or Azathioprine or Methotrexate

MAINTENANCE

MAINTENANCE

Rituximab or Azathioprine or Methotrexate

Treatment based on underlying cause

Treatment based on underlying cause

Treatment based on underlying cause

Treatment based on underlying cause

Additional testing

ANA, anti-dsDNA, anti-Smith, lupus anticoagulant, anticardiolipin antibody. anti-beta2-glycoprotein antibody, C3/C4, RF, anti-CCP anti-Sc170, anti-RNA Pol III, anti-centromere, myositis panel, anti-UT-RNP, ASO titer

Additional testing

ANA, anti-dsDNA, anti-Smith, lupus anticoagulant, anticardiolipin antibody. anti-beta2-glycoprotein antibody, C3/C4, RF, anti-CCP anti-Sc170, anti-RNA Pol III, anti-centromere, myositis panel, anti-UT-RNP, ASO titer

Additional testing

Additional testing

ANA, anti-dsDNA, anti-Smith, lupus anticoagulant, anticardiolipin antibody. anti-beta2-glycoprotein antibody, C3/C4, RF, anti-CCP anti-Sc170, anti-RNA Pol III, anti-centromere, myositis panel, anti-UT-RNP, ASO titer

LV
APAS
RV
SSc
MCTD

LV
APAS
RV
SSc
MCTD





LV

IgAV
CV
BD
PM/DM
PSGN

IgAV
CV
BD
PM/DM
PSGN





IgAV

Clinical features of DAH and RPGN


DAH: Hemoptysis, fever, cough, dyspnea, chest pain
RPGN: Malaise, fatigue, decreased UOP, hypertension, edema

Clinical features of DAH and RPGN


DAH: Hemoptysis, fever, cough, dyspnea, chest pain
RPGN: Malaise, fatigue, decreased UOP, hypertension, edema

Clinical features of DAH and RPGN

Clinical features of DAH and RPGN


DAH: Hemoptysis, fever, cough, dyspnea, chest pain
RPGN: Malaise, fatigue, decreased UOP, hypertension, edema


DAH: Hemoptysis, fever, cough, dyspnea, chest pain DAH:
RPGN: Malaise, fatigue, decreased UOP, hypertension, edema RPGN:

Evaluate for RPGN
BUN, Cr, urinalysis; Urine protein/creatinine, renal biopsy

Evaluate for RPGN
BUN, Cr, urinalysis; Urine protein/creatinine, renal biopsy

Evaluate for RPGN
Evaluate for RPGN

Evaluate for DAH
CBC, coags: CXR; HRCT chest; Bronchoscopy

Evaluate for DAH
CBC, coags: CXR; HRCT chest; Bronchoscopy

Evaluate for DAH
Evaluate for DAH