scholarly journals Pulmonary-Renal Syndrome with Negative ANCAs and Anti-GBM Antibody

2013 ◽  
Vol 2013 ◽  
pp. 1-6 ◽  
Author(s):  
Hiroshi Yamaguchi ◽  
Atsuhisa Shirakami ◽  
Takashi Haku ◽  
Takashige Taoka ◽  
Yoshikazu Nakanishi ◽  
...  

We report the case of a 76-year-old woman who was referred to our hospital for a gradually worsening cough and renal dysfunction. Although pneumonia was initially suspected, imaging findings of the lungs revealed diffuse alveolar hemorrhage at a later date. Renal failure developed and hemodiafiltration was performed on the 9th day. Rapidly progressive glomerulonephritis with crescent formation was diagnosed by renal biopsy. This case presentation has important clinical implications because uncategorizable pulmonary-renal syndrome (PRS) without the presence of ANCAs and anti-GBM antibody is extremely rare and has high rates of morbidity and mortality. No treatment has been established.

2016 ◽  
Vol 2016 ◽  
pp. 1-4
Author(s):  
M. Fröhlich-Gildhoff ◽  
W. J. Jabs ◽  
C. Berhold ◽  
M. K. Kuhlmann ◽  
U. Ketterer ◽  
...  

Purpose. Pulmonary-renal syndrome (PRS) is characterized by diffuse alveolar hemorrhage and rapidly progressive glomerulonephritis mainly due to autoimmune etiologies. Seronegative PRS is a challenging entity to the clinician, since early diagnosis may be missed leading to delayed appropriate treatment. Materials and Methods. We present the clinical course of a 77-year-old patient who was admitted under the suspected diagnosis of pneumogenic sepsis and septic renal failure with fever, dyspnea, and elevated CRP levels. The diagnosis of pulmonary-renal syndrome was initially missed because of the absence of autoantibodies in all serological findings. Results. Despite delayed initiation of immunosuppressive therapy and a prolonged period of dialysis and extracorporeal membrane oxygenation the patient recovered well and was released to a rehabilitation center with nearly normalized creatinine levels. The diagnosis of PRS was established by renal biopsy. Conclusion. This case illustrates the important differential diagnosis of seronegative pulmonary-renal syndrome in patients with pulmonary and renal impairment.


2012 ◽  
Vol 2012 ◽  
pp. 1-4 ◽  
Author(s):  
Tariq Javed ◽  
Parag Vohra

We are presenting a case of renal failure with anti-GBM and p-ANCA antibodies positive. Patients with dual antibodies are considered to be a vasculitis-variant of anti-GBM antibody nephritis. These patients may have atypical presentation and it may delay diagnosis and treatment. Recurrence rate is higher in these patients. We reviewed the literature of cases and studies on cresenteric glomerulonephritis with anti-GBM and p-ANCA positive patients. We recommend that patients suspected with pulmonary-renal syndrome should be checked for anti-GBM and p-ANCA antibodies, should undergo renal biopsy and should should have close long term follow up to watch for recurrence.


2018 ◽  
Vol 2018 ◽  
pp. 1-4
Author(s):  
Bryan Yong ◽  
David A. Power

Background. Pulmonary-renal syndrome is characterised by acute kidney injury, haematuria, and haemoptysis and is a well-recognised presentation of diseases such as ANCA vasculitis that require urgent immunosuppression. Case Presentation. A patient presented with a brief history of haemoptysis, acute renal failure, microscopic haematuria, and severe hypertension. The diagnosis was initially not clear so he was treated with antihypertensives, renal replacement therapy, and immunosuppression. Renal biopsy subsequently showed evidence of malignant hypertension. Autoantibodies were uniformly negative. Conclusions. This case demonstrates that malignant hypertension can present as pulmonary-renal syndrome.


2020 ◽  
Vol 8 (T1) ◽  
pp. 542-547
Author(s):  
Biljana Gerasimovska-Kitanovska ◽  
Gjulshen Selim ◽  
Zvezdana Petronijevik ◽  
Blagica Pecanova ◽  
Gjorgi Stojchev ◽  
...  

BACKGROUND: Antineutrophil cytoplasmatic antibody (ANCA)-associated vasculitis (AAV) has a predilection for the kidney and more than three quarters of patients have renal involvement with rapidly progressive glomerulonephritis. Small-vessel systemic vasculitis may present as pulmonary-renal syndrome and is characterized by necrotizing glomerulonephritis and pulmonary hemorrhage. Diagnosis and therapy for AAV in coronavirus disease (COVID) COVID-19 pandemic require multi-disciplinary collaboration due to the affection of multiple systems and risks associated with immunosuppressive medications. CASE REPORT: A 69-year-old non-smoker, non-diabetic female presented in the outpatient unit at the department of pulmonology with dry cough, malaise, and sub-febrile temperature, lasting for 1 month. The patient had a high suspicion of severe pulmonary-renal syndrome, ANCA-AAV, and acute renal failure requiring hemodialysis. She was treated with corticosteroids, cyclophosphamide, and plasma exchange. The treatment led to temporary improvement. Infections with COVID-19, Enterococcus in the urine, and Acinetobacter in the tracheal aspirate further complicated the clinical picture and despite antibiotic treatment, use of tocilizumab and convalescent plasma, the outcome was lethal. CONCLUSION: It is important to establish the diagnosis and distinguish accurately between vasculitis and infection to provide adequate and timely therapy.


2011 ◽  
Vol 16 (1) ◽  
pp. 173-179 ◽  
Author(s):  
Atsuko Y. Higashi ◽  
Fumiaki Nogaki ◽  
Isoroku Kato ◽  
Takahiko Ono ◽  
Atsushi Fukatsu

2002 ◽  
Vol 103 (s2002) ◽  
pp. 434S-437S ◽  
Author(s):  
Masanori TAKAOKA ◽  
Mikihiro YUBA ◽  
Toshihide FUJII ◽  
Mamoru OHKITA ◽  
Yasuo MATSUMURA

We investigated whether the treatment with 17β-oestradiol has renal protective effects in male rats with ischaemic acute renal failure (ARF). We also examined if the effect of 17β-oestradiol is accompanied by suppression of enhanced endothelin-1 production in postischaemic kidneys. Ischaemic ARF was induced by clamping the left renal artery and vein for 45min followed by reperfusion, 2 weeks after contralateral nephrectomy. Renal function parameters such as blood urea nitrogen, plasma creatinine and creatinine clearance were measured to test the effectiveness of the steroid hormone. Renal function in ARF rats markedly decreased 24h after reperfusion. The ischaemia/reperfusion-induced renal dysfunction was dose-dependently improved by pretreatment with 17β-oestradiol (20 or 100µg/kg, intravenously). Histopathological examination of the kidney of untreated ARF rats revealed severe lesions, such as tubular necrosis, proteinaceous casts in tubuli and medullary congestion, all of which were markedly improved by the higher dose of 17β-oestradiol. In addition, endothelin-1 content in the kidney after the ischaemia/reperfusion increased significantly by approx. 2-fold over sham-operated rats, and this elevation was dose-dependently suppressed by the 17β-oestradiol treatment. These results suggest that oestrogen exhibits protective effects against renal dysfunction and tissue injury induced by ischaemia/reperfusion, possibly through the suppression of endothelin-1 overproduction in postischaemic kidneys.


PEDIATRICS ◽  
1994 ◽  
Vol 93 (4) ◽  
pp. 693-693
Author(s):  
Sara C. McIntire ◽  
Ronald C. Rubenstein ◽  
J. Carlton Gartner ◽  
Nisan Gilboa ◽  
Demetrius Ellis

The patient described by Wattad et al is quite similar to the two children we describe.1 Once again the striking features are acute flank pain and nonoliguric renal dysfunction. The renal biopsy demonstrates mild interstitial nephritis. We believe that knowledge of the association of this clinical syndrome with nonsteroidal anti-inflammatory drug use will make renal biopsy (and its potential complications) unnecessary in evaluating future cases. We strongly suspect that this condition is much more common than previously recognized.


1984 ◽  
pp. 201-204
Author(s):  
Olivier Kourilsky ◽  
Liliane Morel-Maroger ◽  
Gabriel Richet

2018 ◽  
Vol 90 (6) ◽  
pp. 130-136 ◽  
Author(s):  
M L Bulanova ◽  
D V Potapov ◽  
N M Bulanov ◽  
L V Lysenko(Kozlovskaya)

Goodpasture’s disease (anti-GBM disease) is a rare small vessels vasculitis characterized by the presence of autoantibodies directed against the glomerular basement membrane (GBM) and alveolar basement membrane. Common feature of anti-GBM disease is a combination of rapidly progressive glomerulonephritis and alveolar hemorrhage (pulmonary-renal syndrome). We present a case of atypical disease course in a young male patient who developed alveolar hemorrhage without renal failure. The only symptom of renal involvement was isolated hematuria. Plasmapheresis combined with immunosuppression (cyclophosphamide and corticosteroids) was effective. We present a review of state-of-art data on the pathogenesis and disease course of anti-GBM disease.


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