304Lu Wegener’s granulomatosis with Polyangiitis Pulmonary Hemorrhage

19-year-old white male with no significant past history complaints of chest pain and pneumonia diagnosed by outside position and also hemoptysis for the past 3 days. Associated symptoms included myalgias and joint pains. His urine has become tea colored.

 On examination his temperature was 100.7 and his heart rate was 110 beats per minute with a respiratory rate of 20 per minute 

His chest x-ray shows the diffuse bilateral consolidations involving both the upper and lower lobes.

PULMONARY HEMORRHAGE – WEGENER”S GRANULOMATOSIS aka GRANULOMATOSIS WITH POLYANGIITIS, GPA.
The chest x-ray shows diffuse bilateral lobar infiltrates sparing the hilar regions.
Lab shows ANCA positivity, acute renal failure (creatinine 6) and renal biopsy showing crescentic glomerulonephritis. Diagnosis consistent with GPA
Treated with cyclophosphamide
Ashley Davidoff MD TheCommonVein.net 139179

Labs His white cell count was 13.5 and his a hematocrit  was 31. His platelet count was 430,000

His  BUN and his creatinine was 4.1

 Subsequent clinical course showed deterioration in renal function.  Creatinine  increased to 5. He was empirically treated on prednisone. 

Renal biopsy showed a crescentic glomerulonephritis

The presence of anti-neutrophil cytoplasmic antibody to proteinase 3 was diagnostic of Wegener’s granulomatosis aka GPA – Granulomatosis with Polyangiitis

CT scan Acinar Nodules Subpleural Sparing

PULMONARY HEMORRHAGE – WEGENER”S GRANULOMATOSIS aka GRANULOMATOSIS WITH POLYANGIITIS, GPA
19 year old male previously well with history of hemoptysis, sweating, fevers, myalgias, arthritis over 3 weeks.
CT scan scout (above ) shows diffuse bilateral lobar infiltrates with subpleural sparing 
Coronal CT shows bilateral symmetrical lobar nodular consolidations involving the upper and lower lobes.  The upper lobes are more consolidative and the lower lobes have an acinar pattern. These finding are consistent with acute pulmonary hemorrhage
Lab shows ANCA positivity, acute renal failure (creatinine 6) and renal biopsy showing crescentic glomerulonephritis.  Treated with cyclophosphamide
Ashley Davidoff MD TheCommonVein.net 139195c
PULMONARY HEMORRHAGE – WEGENER”S GRANULOMATOSIS aka GRANULOMATOSIS WITH POLYANGIITIS, GPA
19 year old male previously well with history of hemoptysis, sweating, fevers, myalgias, arthritis over 3 weeks.
CT scan in the axial projection shows diffuse bilateral  nodular consolidations (acinar pattern) with subpleural sparing consistent with pulmonary hemorrhage
Lab shows ANCA positivity, acute renal failure (creatinine 6) and renal biopsy showing crescentic glomerulonephritis. Treated with cyclophosphamide
Ashley Davidoff MD TheCommonVein.net 139193c
PULMONARY HEMORRHAGE – WEGENER”S GRANULOMATOSIS aka GRANULOMATOSIS WITH POLYANGIITIS, GPA
19 year old male previously well with history of hemoptysis, sweating, fevers, myalgias, arthritis over 3 weeks.
CT scan in the axial projection shows diffuse bilateral  nodular consolidations (acinar pattern ringed in red) with subpleural sparing consistent with pulmonary hemorrhage
Lab shows ANCA positivity, acute renal failure (creatinine 6) and renal biopsy showing crescentic glomerulonephritis. These finding are consistent with a diagnosis of GPA.  He was  treated with cyclophosphamide
Ashley Davidoff MD TheCommonVein.net 139193cL
PULMONARY HEMORRHAGE – WEGENER”S GRANULOMATOSIS aka GRANULOMATOSIS WITH POLYANGIITIS, GPA
19 year old male previously well with history of hemoptysis, sweating, fevers, myalgias, arthritis over 3 weeks.
CT scan in the axial projection shows diffuse bilateral  nodular consolidations ( with subpleural sparing consistent with pulmonary hemorrhage.  Air bronchograms  are noted in the posterior segment of the right upper lobe  and the superior segment of the right lower lobe (magnified in the lower image
Labs showed ANCA positivity, acute renal failure (creatinine 6) and renal biopsy showed crescentic glomerulonephritis. These findings are consistent with a diagnosis of GPA.  He was  treated with cyclophosphamide
Ashley Davidoff MD TheCommonVein.net 139185c
PULMONARY HEMORRHAGE – WEGENER”S GRANULOMATOSIS aka GRANULOMATOSIS WITH POLYANGIITIS, GPA
19 year old male previously well with history of hemoptysis, sweating, fevers, myalgias, arthritis over 3 weeks.
CT scan in the axial projection shows diffuse bilateral  nodular consolidations  with subpleural sparing consistent with pulmonary hemorrhage.  Air bronchograms  are noted in the posterior segment of the right upper lobe (b ringed in c)   and the superior segment of the right lower lobe  (b ringed in c)
Labs showed ANCA positivity, acute renal failure (creatinine 6) and renal biopsy showed crescentic glomerulonephritis. These finding are consistent with a diagnosis of GPA.  He was  treated with cyclophosphamide
Ashley Davidoff MD TheCommonVein.net 139185cL