A rare lung manifestation with rapidly progressive glomerulonephritis (RPGN)

Farah, Raymond; Vaisban, Eleonora; Geron, Ronit; Cohen, Hector; Nassar, Faris

doi:10.1016/j.ejim.2004.09.010

Cited by 3 publications

(1 citation statement)

References 4 publications

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“…[ 9 , 10 ] Eisenberger et al [ 8 ] reported pulmonary involvement in 3 out of 20 patients with ANCA negative pauci immune renal vasculitis of which 2 had cavitary nodules. Farah et al [ 11 ] reported one case of idiopathic bronchiolitis obliterans organizing pneumonia (BOOP) associated with pauci-immune renal vasculitis that terminated in rapidly progressive glomerulonephritis (RPGN). However, association of diffuse alveolar hemorrhage with ANCA negative glomerulonephritis is rare.…”

Section: Discussionmentioning

confidence: 99%

Pulmonary renal syndrome

Saladi

Shaikh²,

Saad³

et al. 2018

Medicine

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Rationale:Pulmonary renal syndrome (PRS) is a term most commonly used to describe a combination of glomerulonephritis and pulmonary hemorrhage as a manifestation of a multisystem autoimmune disease. It is usually associated with ANCA vasculitis and anti-GBM disease. Diffuse alveolar hemorrhage in a patient with ANCA and anti-GBM negative pauci-immune glomerulonephritis is rare and optimal management is unknown.Patient concerns:An 85-year-old man with hypertension, diabetes mellitus, prostate cancer and recently diagnosed pauci-immune necrotizing glomerulonephritis presented to our emergency department with worsening dyspnea and pedal edema for several days. Clinical presentation and radiological studies were suggestive of fluid overload but he developed worsening respiratory failure despite hemodialysis.Diagnoses:Bronchoscopy confirmed diffuse alveolar hemorrhage. ANCA and anti-GBM antibodies were negative. The patient was diagnosed with pulmonary renal syndrome – diffuse alveolar hemorrhage in the setting of ANCA and anti-GBM negative pauci-immune glomerulonephritis.Interventions:Patient was started on intravenous pulse steroids, cyclophosphamide and received seven sessions of plasmapheresis.Outcomes:There was an improvement in patient's respiratory status and repeat bronchoscopy at the end of treatment did not show diffuse alveolar hemorrhage.Lessons:Pauci-immune crescentic necrotizing glomerulonephritis is usually associated with the presence of ANCA, however, ANCA may be absent in 10% of these cases. Immunosuppression is the mainstay of treatment for ANCA and anti-GBM associated PRS. This case highlights the importance of immunosuppression and plasmapheresis in patients with ANCA negative vasculitis due to presence of unidentified serum antibodies. If left untreated, these patients can have a fulminant course with high mortality ranging from 25 to 50%.

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Section: Discussionmentioning

confidence: 99%

Pulmonary renal syndrome

Saladi

Shaikh²,

Saad³

et al. 2018

Medicine

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Diffuse alveolar haemorrhage in ANCA-negative pauci-immune crescentic glomerulonephritis

Sandhu¹,

Casares²,

Farias³

et al. 2010

Clinical Kidney Journal

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Pulmonary renal syndrome (PRS) is a combination of diffuse pulmonary haemorrhage and glomerulonephritis (GN). Though an established form of presentation in anti-neutrophil cytoplasmic autoantibody (ANCA)-associated GN and vasculitis, diffuse pulmonary haemorrhage is extremely unusual in those with ANCA-negative GN. We present here a case of a 76-year-old Hispanic female with stage IV chronic kidney disease (serum creatinine of 2 mg/dL), who presented with diffuse alveolar haemorrhage and nephritic syndrome. Less than 1 week prior to the full-blown PRS, she was treated for an apparent pneumonia as was evidenced by a right lower lobe infiltrate on her chest X-ray. Retrospectively, this was likely a focal pulmonary haemorrhage. ANCA were persistently negative, and the remainder of her immunologic workup was normal. Renal biopsy was diagnostic of crescentic pauci-immune GN. The patient required a ventilator and haemodialysis support (serum creatinine 6 mg/dL), and was successfully treated with methylprednisolone, cyclophosphamide and a total of six cycles of plasmapheresis. Once her oliguria resolved, the creatinine plateaued at 2.7 mg/dL. Our case illustrates that diffuse alveolar haemorrhage can be a distinct clinical feature even in patients with ANCA-negative pauci-immune crescentic glomerulonephritis.

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