Clinico-pathological considerations in a 48-years-old female with acute kidney injury: is it lupus nephritis, ANCA-associated vasculitis or something else?

The relationship between kidneys and anticoagulation is complex, especially after introduction of the direct oral anticoagulants (DOAC). It is recently growing evidence of an anticoagulant-related nephropathy (ARN), a form of acute kidney injury caused by excessive anticoagulation. The pathogenesis of kidney damage in this setting is multifactorial, and nowadays, there is no established treatment. We describe a case of ARN, admitted to our Nephrology Unit with a strong suspicion of ANCA-associated vasculitis due to gross haematuria and haemoptysis; the patient was being given dabigatran. Renal biopsy excluded ANCA-associated vasculitis and diagnosed a red blood cell cast nephropathy superimposed to an underlying IgA nephropathy. Several mechanisms are possibly responsible for kidney injury in ARN: tubular obstruction, cytotoxicity of heme-containing molecules and free iron, and activation of proinflammatory/proﬁbrotic cytokines. Therefore, the patient was given a multilevel strategy of treatment. A combination of reversal of coagulopathy (i.e., withdrawal of dabigatran and infusion of its specific antidote) along with administration of fluids, sodium bicarbonate, steroids, and mannitol resulted in conservative management of AKI and fast recovery of renal function. This observation could suggest a prospective study aiming to find the best therapy of ARN.

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Renal biopsy teaching case: A patient with scleroderma, hypertension, acute kidney injury and PR3 ANCA positivity

Journal of Clinical Images and Medical Case Reports ◽

10.52768/2766-7820/1168 ◽

2021 ◽

Vol 2 (3) ◽

Author(s):

Sam Kant ◽

◽

Avi Rosenberg ◽

Fred Wigley ◽

Duvuru Geetha D ◽

...

Keyword(s):

Acute Kidney Injury ◽

Renal Biopsy ◽

Kidney Injury ◽

Scleroderma Renal Crisis ◽

Disease Course ◽

Common Cause ◽

Teaching Case ◽

Anca Associated Vasculitis ◽

Renal Crisis

Scleroderma Renal Crisis (SRC) is the most common cause of Acute Kidney Injury (AKI) in scleroderma and occurs early during the disease course while ANCA Associated Vasculitis (AAV) is a rare cause of acute kidney injury occurring late in the disease course. We report a case of AKI with positive PR3 ANCA serology late in the disease course with a renal biopsy revealing findings of SRC.

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134 OUTCOME OF SEVERE LUPUS NEPHRITIS WITH ACUTE KIDNEY INJURY TREATED WITH THERAPEUTIC PLASMAPHERESIS

Kidney International Reports ◽

10.1016/j.ekir.2017.06.115 ◽

2017 ◽

Vol 2 (4) ◽

pp. S35-S36

Author(s):

A.S. Shahnon ◽

Y. Suryati ◽

H.S. Wong

Keyword(s):

Acute Kidney Injury ◽

Lupus Nephritis ◽

Kidney Injury ◽

Severe Lupus Nephritis

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Severe Complications from an Unexpectedly High Serum Mycophenolic Acid Concentration in a Patient with Renal Failure Secondary to Lupus Nephritis: A Case Report

Case Reports in Nephrology and Dialysis ◽

10.1159/000500516 ◽

2019 ◽

Vol 9 (2) ◽

pp. 72-78 ◽

Cited By ~ 1

Author(s):

Yuji Doi ◽

Hirotsugu Kitayama ◽

Masayoshi Yamada ◽

Yudai Miyama

Keyword(s):

Acute Kidney Injury ◽

Renal Failure ◽

Mycophenolate Mofetil ◽

Lupus Nephritis ◽

Acid Concentration ◽

Immunosuppressive Agents ◽

Kidney Injury ◽

Immunosuppressive Drugs ◽

High Serum ◽

Symptom Relapse

Mycophenolate mofetil (MMF) is used widely to treat lupus nephritis and is considered safer than other immunosuppressive drugs. Reports on severe complications related to MMF are sparse. We report a case of a patient with lupus nephritis in whom severe complications were possibly caused by MMF. The patient was a 17-year-old girl who received a diagnosis of lupus nephritis at the age of 14 years and had been taking steroid and immunosuppressive agents since then. One week after starting MMF 1 g/day instead of mizoribine owing to symptom relapse and serologic data deterioration, she presented with seizure, accompanied by leukopenia, thrombocytopenia, and renal failure. We discontinued MMF because she had extremely high serum mycophenolate acid concentration (88 µg/mL). A few weeks later, she recovered without any complications and was discharged. Although rare, clinicians should be aware that serum mycophenolate acid concentration may become extremely high in the setting of acute kidney injury. In such circumstances, they should perform serum concentration monitoring to avoid possible adverse events.

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ANCAassociated vasculitis in a man with scleroderma and pulmonary fibrosis

Canadian Journal of General Internal Medicine ◽

10.22374/cjgim.v13i3.164 ◽

2018 ◽

Vol 13 (3) ◽

pp. 21-24

Author(s):

Alyson Wong ◽

Christine McDonald ◽

John Thenganatt

Keyword(s):

Acute Kidney Injury ◽

Pulmonary Hypertension ◽

Interstitial Lung Disease ◽

Lung Disease ◽

Kidney Injury ◽

Internal Organs ◽

Anca Associated Vasculitis ◽

Pulmonary Manifestations ◽

Pulmonary Arterial ◽

New Diagnosis

Systemic sclerosis, or scleroderma, is a connective tissue disease that causes fibrosis of the skin and potentially internal organs (1). The most common lung findings in those with scleroderma are interstitial lung disease and pulmonary hypertension (1). Here we describe a 58-year-old man with scleroderma, interstitial lung disease and pulmonary arterial hypertension. He presented with atypical pulmonary manifestations and an acute kidney injury caused by a new diagnosis of ANCA-associated vasculitis.

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Call for action in ANCA-associated vasculitis and lupus nephritis: promises and challenges of SGLT-2 inhibitors

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2021-221474 ◽

2021 ◽

pp. annrheumdis-2021-221474

Author(s):

Marcus Säemann ◽

Andreas Kronbichler

Keyword(s):

Lupus Nephritis ◽

Molecular Mechanisms ◽

Cardiovascular Health ◽

Kidney Injury ◽

Renal Outcome ◽

Diabetic Patients ◽

Clinical Effects ◽

Cardiovascular Outcome Trials ◽

Anca Associated Vasculitis ◽

Risk Patients

Sodium–glucose cotransporter- 2 inhibitors (SGLT- 2i) have recently been demonstrated to exert profound cardio- and nephroprotection in large cardiovascular outcome trials. They reduce progression of chronic kidney disease (CKD) including albuminuria and improve outcomes in heart failure patients with and without type 2 diabetes on top of angiotensin-blocking agents. These benefits translate into improved mortality in cardiorenal risk patients. While the detailed molecular mechanisms underlying these surprising clinical outcomes are not fully understood, their antidiabetic properties are not causative. Rather reduction of glomerular hyperfiltration and tubuloprotection are involved as root cause mechanisms of their clinical effects. Finally, their side effect profile is advantageous especially in non-diabetic patients also reducing the risk of acute kidney injury. Among the independent risk factors for excess mortality, CKD is still one of the strongest predictors of a poor prognosis in patients with both ANCA- associated vasculitis (AAV) and lupus nephritis (LN). Since patients with autoimmune disease were excluded from all recent large renal outcome trials with SGLT-2i and given their strong nephroprotective potential, we herein advocate to study this unique class of disease-modifying therapies when it comes to kidney and cardiovascular health in patients with AAV and LN.

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Rapidly Progressive Glomerulonephritis Secondary to IgA Nephropathy in a Patient with Systemic Lupus Erythematosus

Case Reports in Nephrology ◽

10.1155/2019/8354823 ◽

2019 ◽

Vol 2019 ◽

pp. 1-7 ◽

Cited By ~ 1

Author(s):

Amol M. Patel ◽

Lily Anne Romero Karam ◽

Stephanie C. Fuentes Rojas ◽

Warren E. Redfearn ◽

Luan D. Truong ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Acute Kidney Injury ◽

Lupus Nephritis ◽

Iga Nephropathy ◽

Lupus Erythematosus ◽

Kidney Injury ◽

Disease Stage ◽

Immunofluorescent Staining ◽

Renal Diseases ◽

Systemic Lupus

Lupus nephritis is a common manifestation of systemic lupus erythematosus (SLE). IgA nephropathy is a common type of primary glomerulonephritis. Renal manifestations in SLE patients are often due to lupus nephritis; however, renal diseases unrelated to lupus nephritis are rarely reported. While crescentic IgA nephropathy with rapid clinical progression is rare, its development in patients with SLE in the absence of lupus nephritis is even more unusual. A 74-year-old woman with a history of SLE without known renal involvement, chronic kidney disease stage IIIa, congestive heart failure, hypertension, and type 2 diabetes mellitus presented with acute kidney injury. Her creatinine continued to rise rapidly. Renal biopsy revealed mesangial proliferative glomerulonephritis with crescent formation. Immunofluorescent staining showed IgA and C3 mesangial deposition and absence of C4 and C1q, consistent with IgA nephropathy. She received a course of methylprednisolone and plasmapheresis. Unfortunately, her renal function continued to deteriorate, and she was started on hemodialysis which was continued after hospital discharge. This case illustrates crescentic IgA nephropathy without lupus nephritis as the cause of acute kidney injury in a patient with SLE. It highlights the observation that renal diseases other than lupus nephritis can develop in SLE patients.

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Checkpoint inhibitor-related renal vasculitis and use of rituximab

Journal for ImmunoTherapy of Cancer ◽

10.1136/jitc-2020-000750 ◽

2020 ◽

Vol 8 (2) ◽

pp. e000750 ◽

Cited By ~ 1

Author(s):

Omar Mamlouk ◽

Jamie S Lin ◽

Maen Abdelrahim ◽

Amanda S Tchakarov ◽

William F Glass ◽

...

Keyword(s):

Acute Kidney Injury ◽

Checkpoint Inhibitor ◽

Treatment Options ◽

Kidney Injury ◽

Comprehensive Cancer Center ◽

Cancer Center ◽

Lung Involvement ◽

Patients With Cancer ◽

Anca Associated Vasculitis ◽

Renal Vasculitis

The percentage of patients with cancer eligible for checkpoint inhibitor (CPI) therapy has increased rapidly over the past few years and approaches 45%. As a result, more cases of CPI-related nephrotoxicity, including a rare subset with vasculitis, are being reported. To elucidate the clinical presentation of CPI-associated renal vasculitis and its possible mechanisms, treatment options and prognosis, we describe cases from a comprehensive cancer center and reviewed the literature for similar cases. We retrospectively reviewed the charts of all patients with cancer from 2014 to 2020 who were diagnosed with CPI-related nephrotoxicity and underwent a kidney biopsy. We identified five cases of renal vasculitis: three patients were diagnosed with seronegative antineutrophil cytoplasm antibody (ANCA)-associated vasculitis, one case with seropositive ANCA-associated vasculitis and one case was diagnosed with IgA vasculitis. Of these cases, four patients were receiving nivolumab, and one patient was receiving tremelimumab. All patients had microscopic hematuria, four out of five patients had negative ANCA serology, one patient had concurrent lung involvement and positive ANCA serology, and all had severe acute kidney injury with creatinine >4.50 mg/dL on diagnosis. All patients were treated by discontinuing CPI and initiating corticosteroids and rituximab. Three patients received plasmapheresis; two of these required renal replacement therapy including the patient with lung involvement. All patients after rituximab had a partial or complete renal response. Two patients died within 8 months of diagnosis due to malignancy progression. None of the patients had a relapse of vasculitis. We demonstrated that CPI can be associated with different types of renal vasculitis that are predominantly ANCA negative and manifest as severe acute kidney injury. Despite the lack of strong evidence, treatment similar to treatment of primary seropositive ANCA-associated vasculitis with corticosteroids and rituximab is well tolerated with favorable renal outcomes.

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