scholarly journals Crescentic poststreptococcal acute glomerulonephritis accompanied by small vessel vasculitis: case report of an elderly male

2019 ◽  
Vol 20 (1) ◽  
Author(s):  
Keiko Yano ◽  
Hiroyuki Suzuki ◽  
Takashi Oda ◽  
Yoshihiko Ueda ◽  
Tatsuo Tsukamoto ◽  
...  
Keyword(s):  
Renal Function ◽  
Small Vessel ◽  
Immunofluorescent Staining ◽  
Small Vessel Vasculitis ◽  
Rapid Decline ◽  
Acute Glomerulonephritis ◽  
Plasmin Activity ◽  
Elderly Male ◽  
Necrotizing Glomerulonephritis ◽  
Poststreptococcal Acute Glomerulonephritis

Abstract Background Poststreptococcal acute glomerulonephritis (PSAGN) in the elderly tends to have a severe clinical course and often presents with crescentic necrotizing glomerulonephritis in the renal biopsy. However, vasculitis lesions are unusual. Case presentation We present a 71-year-old man who was admitted to our hospital for a recurrent gout attack with a rapid decline of renal function. Low C3 levels and a high anti-streptolysin O titer were observed, while myeloperoxidase- and proteinase 3- antineutrophil cytoplasmic antibody (ANCA) were negative. In addition to cellular crescent and necrosis lesions, diffuse peritubular capillaritis and venulitis as well as small arteriole vasculitis in the glomerular hilus were also apparent. Although granular C3c deposits in the capillary wall and hump lesions were not found, immunofluorescent staining for nephritis-associated plasmin receptor (NAPlr) and in situ zymography for plasmin activity were both positive. We thus diagnosed PSAGN accompanied by small vessel vasculitis. Steroid therapy gradually improved the patient’s renal function, and hemodialysis was discontinued after 1 month. Conclusions In our case, streptococcus infection might have concurrently provoked vasculitis, and NAPlr staining was useful for confirming diagnosis.

scholarly journals P0475CRESCENTIC GLOMERULONEPHRITIS FOLLOWING HCV TREATMENT WITH DAAS, THE NON-CLASSIC TALE

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Mohamed Elrggal ◽  
Mariam Emam ◽  
Wesam Ismail
Keyword(s):  
Acute Kidney Injury ◽  
Serum Creatinine ◽  
Sustained Viral Response ◽  
Kidney Injury ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
Hcv Treatment ◽  
Drug Induced ◽  
First Case ◽  
Necrotizing Glomerulonephritis

Abstract Background and Aims Observational studies suggest that acute kidney injury may occur in up to 15% of patients treated with sofosbuvir based regimens. Histological findings show features of acute interstitial nephritis (AIN) or acute tubular necrosis (ATN). Here, we report the first case of small vessel vasculitis following sofosbuvir treatment. Method A 65-year-old female with controlled T2DM was recently diagnosed with HCV. She attained sustained viral response (SVR) after a three-month course of (sofosbuvir + daclatasvir + ribavirin). Kidney functions were normal pre and post treatment. Three months later, she presented with puffiness, bilateral lower extremities edema (no rash) and vomiting. Labs showed acute kidney injury (AKI), nephrotic proteinuria and haematuria (table 1). Immunological investigations (C3, C4, ANA, ANCA and anti-GBM), paraproteinemia workup and cryoglobulins were all negative. Renal US was also normal. Kidney biopsy revealed focal necrotizing glomerulonephritis with 70% crescents (figure 3,4). No chronic changes were detected in the glomeruli, interstitium or tubules. The patient received pulse methylprednisolone 0.5 gm for 3 days followed by oral prednisolone 60 mg/day. Oral cyclophosphamide was initiated at 150 mg after biopsy result was obtained. Our patient showed clinical and laboratory improvement (figure 1,2), however, she developed bone marrow suppression that required cessation of cyclophosphamide. Valsartan initiated to control proteinuria with slight increase in serum creatinine to 1.4 mg/dl. The patient is still under close follow up every two weeks, with a plan to introduce rituximab if serum creatinine continued to increase. Results AKI following HCV treatment with DAA has been reported. Explanation includes AIN, ATN and cryoglobulinemic vasculitis (CV). AIN and ATN usually occur during the treatment course, which is not the case in our patient as she developed AKI three months following the end of the treatment, and the biopsy did not show signs of either ATN or AIN. New onset CV has been reported previously in 3 case reports following SVR after DAA treatment. Previous reports explained that HCV can induce B-cell clonal proliferation that may persist independent of viral eradication and produce IgM kappa which will result in cryoglobulinemic GN, again this is not true in our case. Our patient did not have a rash, her serum cryoglobulin was negative and complement levels (C3 and C4) were normal and biopsy did not show evidence of CV. As far as we know, this is the first case with suspected sofosbuvir associated small vessel vasculitis to be reported. Despite the patient had a negative serum ANCA levels, we suspect that this is a case of drug induced vasculitis. Drug induced vasculitis has been reported with hydralazine, propylthiouracil and cocaine, none of these drugs were used in our case. Also, there is no reported case of vasculitis associated with ribavirin or daclatasvir. Thus, we suspect Sofosbuvir is the cause of drug induced vasculitis in this patient. Conclusion Crescentic GN following HCV treatment using DAA is a serious complication that should be promptly diagnosed and managed. Physicians treating HCV infected patients should be aware of this possible complication and monitor for kidney function even after achieving SVR.

scholarly journals Glomerular Deposition of Nephritis-Associated Plasmin Receptor (NAPlr) and Related Plasmin Activity: Key Diagnostic Biomarkers of Bacterial Infection-related Glomerulonephritis

2020 ◽  
Vol 21 (7) ◽  
pp. 2595 ◽  
Author(s):  
Takahiro Uchida ◽  
Takashi Oda
Keyword(s):  
Streptococcal Infection ◽  
Group A Streptococcus ◽  
Acute Glomerulonephritis ◽  
Similar Distribution ◽  
Plasmin Activity ◽  
Glomerular Diseases ◽  
Diagnostic Biomarkers ◽  
Group A ◽  
Poststreptococcal Acute Glomerulonephritis ◽  
Similar Distribution Pattern

It is widely known that glomerulonephritis (GN) often develops after the curing of an infection, a typical example of which is GN in children following streptococcal infections (poststreptococcal acute glomerulonephritis; PSAGN). On the other hand, the term “infection-related glomerulonephritis (IRGN)” has recently been proposed, because infections are usually ongoing at the time of GN onset in adult patients, particularly in older patients with comorbidities. However, there has been no specific diagnostic biomarker for IRGN, and diagnosis is based on the collection of several clinical and pathological findings and the exclusion of differential diagnoses. Nephritis-associated plasmin receptor (NAPlr) was originally isolated from the cytoplasmic fraction of group A streptococcus as a candidate nephritogenic protein for PSAGN and was found to be the same molecule as streptococcal glyceraldehyde-3-phosphate dehydrogenase and plasmin receptor. NAPlr deposition and related plasmin activity were observed with a similar distribution pattern in the glomeruli of patients with PSAGN. However, glomerular NAPlr deposition and plasmin activity could be observed not only in patients with PSAGN but also in patients with other glomerular diseases, in whom a preceding streptococcal infection was suggested. Furthermore, such glomerular staining patterns have been demonstrated in patients with IRGN induced by bacteria other than streptococci. This review discusses the recent advances in our understanding of the pathogenesis of bacterial IRGN, which is characterized by NAPlr and plasmin as key biomarkers.

scholarly journals Patient with Antineutrophil Cytoplasmic Antibody Associated Small Vessel Vasculitis, Acute Renal Failure, and Coronavirus Disease-19 Pneumonia: A Diagnostic and Therapeutic Challenge

2020 ◽  
Vol 8 (T1) ◽  
pp. 542-547
Author(s):  
Biljana Gerasimovska-Kitanovska ◽  
Gjulshen Selim ◽  
Zvezdana Petronijevik ◽  
Blagica Pecanova ◽  
Gjorgi Stojchev ◽  
...  
Keyword(s):  
Renal Failure ◽  
Acute Renal Failure ◽  
Systemic Vasculitis ◽  
Renal Involvement ◽  
Rapidly Progressive Glomerulonephritis ◽  
Pulmonary Hemorrhage ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
Necrotizing Glomerulonephritis ◽  
Pulmonary Renal Syndrome

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.

scholarly journals Atypical Bacterial Pathogens and Small-Vessel Leukocytoclastic Vasculitis of the Skin in Children: Systematic Literature Review

Pathogens ◽  
2021 ◽  
Vol 10 (1) ◽  
pp. 31
Author(s):  
Céline Betti ◽  
Pietro Camozzi ◽  
Viola Gennaro ◽  
Mario G. Bianchetti ◽  
Martin Scoglio ◽  
...  
Keyword(s):  
Mycoplasma Pneumoniae ◽  
Legionella Pneumophila ◽  
Leukocytoclastic Vasculitis ◽  
Systemic Vasculitis ◽  
Bacterial Pathogen ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
Systemic Involvement ◽  
Symptomatic Disease ◽  
Atypical Pathogen

Leukocytoclastic small-vessel vasculitis of the skin (with or without systemic involvement) is often preceded by infections such as common cold, tonsillopharyngitis, or otitis media. Our purpose was to document pediatric (≤18 years) cases preceded by a symptomatic disease caused by an atypical bacterial pathogen. We performed a literature search following the Preferred Reporting of Systematic Reviews and Meta-Analyses guidelines. We retained 19 reports including 22 cases (13 females and 9 males, 1.0 to 17, median 6.3 years of age) associated with a Mycoplasma pneumoniae infection. We did not find any case linked to Chlamydophila pneumoniae, Chlamydophila psittaci, Coxiella burnetii, Francisella tularensis, or Legionella pneumophila. Patients with a systemic vasculitis (N = 14) and with a skin-limited (N = 8) vasculitis did not significantly differ with respect to gender and age. The time to recovery was ≤12 weeks in all patients with this information. In conclusion, a cutaneous small-vessel vasculitis with or without systemic involvement may occur in childhood after an infection caused by the atypical bacterial pathogen Mycoplasma pneumoniae. The clinical picture and the course of cases preceded by recognized triggers and by this atypical pathogen are indistinguishable.

scholarly journals POS1120 PRESENCE OF TOPHI IS ASSOCIATED WITH A RAPID DECLINE IN THE RENAL FUNCTION IN PATIENTS WITH GOUT

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 839.1-839
Author(s):  
Y. J. Oh ◽  
K. W. Moon
Keyword(s):  
Renal Function ◽  
Renal Dysfunction ◽  
Clinical Characteristics ◽  
Multivariate Logistic Regression Analysis ◽  
Joint Involvement ◽  
Rapid Decline ◽  
Symptom Duration ◽  
Tophaceous Gout ◽  
Duration Of Symptoms ◽  
Increased Risk

Background:Gout is the most common inflammatory arthritis resulting from a chronic deposition of MSU crystals in the joints and other soft tissues. After the process of repeated tissue damage and repair due to gout, tophi could be formed around the affected joints. Intra-articular tophi may sometimes result in bone destruction, joint deformities, and dysfunction which can adversely affect the patient’s quality of life. Furthermore, early-onset tophaceous gout patients are more likely to develop renal dysfunction, however, few studies have investigated if the presence of tophi is related with the progression of renal dysfunction in gout patients.Objectives:We aimed to compare clinical characteristics of patients with and without tophi at the time of the diagnosis of gout and investigate the effect of tophi on the renal function in gout patients.Methods:Data of 257 patients who were first diagnosed with gout at the Kangwon National University Hospital from January 2012 to December 2018 were retrospectively studied. Patients were divided into 2 groups according to the presence of tophi at the diagnosis. We compared clinical characteristics and the progression of renal dysfunction between the two groups.Results:Of all patients, 66 (25.5%) initially presented with tophi. Patients with tophi were older, had a longer duration of symptoms, and had a higher prevalence of multiple joint involvement than those without tophi. The decline in the eGFR was more prominent in patients with tophi than in those without (-4.8±14.5 ml/min/1.73m2vs. -0.7±11.9 ml/min/1.73m2, respectively; P=0.039). In multivariate logistic regression analysis, a prolonged symptom duration (odds ratio [OR], 1.010; 95% confidence interval [CI], 1.004–1.017; P=0.001) and multiple joint involvement (OR, 3.027; 95% CI, 1.831–5.004; P<0.001) were significantly associated with increased risk of formation of tophi. The presence of tophi was significantly associated with a rapid decline in the eGFR (β=-0.141; P=0.035).Conclusion:A prolonged symptom duration and multiple joint involvement were independent risk factors for tophi as the presenting symptom in gout patients. The presence of tophi was associated with a declining renal function. Therefore, an early diagnosis and active treatment are important in tophaceous gout.References:[1]Bardin T, Richette P. Definition of hyperuricemia and gouty conditions. Curr Opin Rheumatol 2014;26:186-91.[2]Liu F, Du GL, Song N, Ma YT, Li XM, Gao XM, et al. Hyperuricemia and its association with adiposity and dyslipidemia in Northwest China: results from cardiovascular risk survey in Xinjiang (CRS 2008-2012). Lipids Health Dis 2020;19:58.[3]Dalbeth N, Merriman TR, Stamp LK. Gout. Lancet 2016;388:2039-52.[4]Ruoff G, Edwards NL. Overview of Serum Uric Acid Treatment Targets in Gout: Why Less Than 6 mg/dL? Postgrad Med 2016;128:706-15.[5]Bieber A, Schlesinger N, Fawaz A, Mader R. Chronic tophaceous gout as the first manifestation of gout in two cases and a review of the literature. Semin Arthritis Rheum 2018;47:843-8.Disclosure of Interests:None declared.

The Induction of Autoimmunity to MPO in WKY Rats Results in ANCA-Positive Small Vessel Vasculitis

2003 ◽  
Vol 104 (s49) ◽  
pp. 50P-51P
Author(s):  
C.L. Smyth ◽  
J. Smith ◽  
H.T. Cook ◽  
D.O. Haskard ◽  
C.D. Pusey
Keyword(s):  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
Wky Rats
Sign in / Sign up

Export Citation Format

Share Document