Renal Involvement in Systemic Vasculitis

Author(s):  
R.A. Sinico ◽  
E. Sabadini ◽  
R. Boeri ◽  
E. Bonacina
2015 ◽  
Vol 2015 ◽  
pp. 1-5 ◽  
Author(s):  
Lohit Garg ◽  
Sagar Gupta ◽  
Abhishek Swami ◽  
Ping Zhang

Levamisole is an antihelminthic and immunomodulator medication that was banned by the USFDA in 1998. It has been increasingly used to adulterate cocaine due to its psychotropic effects and morphological properties. Adverse reactions including cutaneous vasculitis, thrombocytopenia, and agranulocytosis have been well described. Despite systemic vasculitis in this setting, renal involvement is uncommon. We report here a case of ANCA positive systemic vasculitis with biopsy proven immune complex mediated glomerulonephritis likely secondary to levamisole/cocaine. A 40-year-old Caucasian male with no past medical history presented with 3-week history of fatigue, skin rash, joint pains, painful oral lesions, oliguria, hematuria, worsening dyspnea on exertion, and progressive lower extremity edema. He had a history of regular tobacco and cocaine use. Lab testing revealed severe anemia, marked azotemia, deranged electrolytes, and 4.7 gm proteinuria. Rheumatologic testing revealed hypocomplementemia, borderline ANA, myeloperoxidase antibody, and positive atypical p-ANCA. Infectious and other autoimmune workup was negative. Kidney biopsy was consistent with immune mediated glomerulonephritis and showed mesangial proliferation and immune complex deposition consisting of IgG, IgM, and complement. High dose corticosteroids and discontinuing cocaine use resulted in marked improvement in rash, mucocutaneous lesions, and arthritis. There was no renal recovery and he remained hemodialysis dependent.


2018 ◽  
Vol 38 (9) ◽  
pp. 406-412 ◽  
Author(s):  
Shasha Liu ◽  
Nanfang Li ◽  
Qing Zhu ◽  
Bin Zhu ◽  
Ting Wu ◽  
...  

Author(s):  
Alan D. Salama

Primary systemic vasculitis frequently leads to renal involvement and is responsible for significant numbers of patients progressing to end-stage renal disease. Frequently this is due to small vessel vasculitis, in association with antineutrophil cytoplasm antibody, which requires prompt recognition and timely therapeutic intervention to optimize renal and patient outcomes. Other organ systems are often affected. Relapses occur in about 50%.Less commonly medium or larger vessel vasculitis may involve the kidneys and through ischaemia lead to impaired renal function and renovascular hypertension, as in Takayasu’s or Kawasaki disease, and polyarteritis nodosa (PAN).


2010 ◽  
Vol 25 (10) ◽  
pp. 3307-3314 ◽  
Author(s):  
S. Laurino ◽  
A. Chaudhry ◽  
A. Booth ◽  
G. Conte ◽  
D. Jayne

2021 ◽  
Vol 2021 ◽  
pp. 1-8
Author(s):  
Xiayire Aierken ◽  
Qing Zhu ◽  
Ting Wu ◽  
Sha sha Liu ◽  
Yuan yuan Cao ◽  
...  

Objectives. Systemic vasculitis includes a group of disorders characterized by inflammation of the vessel wall, involving multiple systems, and can cause malignant hypertension. CD163 is a specific marker of anti-inflammatory macrophages. This study is aimed at evaluating the CD163 levels in relation to systemic vasculitis and renal involvements. Methods. Urinary CD163 levels were retrospectively measured by enzyme-linked immunosorbent assay (ELISA) in 51 patients with systemic vasculitis, 42 essential hypertensions, and 36 healthy volunteers. The associations between urinary CD163 levels and clinical indicators were analyzed. Results. Urinary CD163 levels were significantly higher in patients with systemic vasculitis [68.20 (38.25~158.78) (pg/ml)] compared to essential hypertension [43.86 (23.30-60.71) (pg/ml)] ( p = 0.003 ) and the healthy volunteers [30.76 (9.30-54.16) (pg/ml)] ( p < 0.001 ). Furthermore, systemic vasculitis patients with renal involvement had significantly higher urinary CD163 levels relative to patients without renal involvement [86.95 (47.61 and 192.38) pg/ml] vs. [41.99 (17.70 and 71.95) pg/ml, p = 0.005 ]. After control factors age, sex, and BMI, urinary CD163 levels in systemic vasculitis patients were positively correlated with serum creatinine, blood urea nitrogen, and β-2 microglobulin ( r = 0.45 , 0.48, and 0.46; p = 0.001 , 0.001, and 0.002, respectively). In addition, we found the level of urinary CD163 in granulomatous vasculitis (including TA, GPA, and EGPA) was significantly higher than that in necrotizing vasculitis (including PAN) [86.95 (41.99 and 184.82) pg/ml] vs. [45.73 (21.43 and 74.43) pg/ml, p = 0.016 ]. Conclusion. Urinary CD163 levels were significantly higher in patients with systemic vasculitis, especially in patients with renal involvement. Thus, urinary CD163 has the potential to be a biomarker for systemic vasculitis with renal involvement.


2022 ◽  
Vol 23 (1) ◽  
Author(s):  
Hanui Park ◽  
Miji Lee ◽  
Jin Seon Jeong

Abstract Background Immunoglobulin A vasculitis (IgA vasculitis) is one of the most common forms of vasculitis in children. It rarely occurs in adults. It is a systemic vasculitis with IgA deposition and is characterized by the classical tetrad of purpura, arthritis/arthralgia, gastrointestinal and renal involvement. Certain types of infections, and pharmacological agents have been reported to be associated with IgA vasculitis. Here, we describe a case of IgA vasculitis triggered by infective endocarditis in a patient undergoing maintenance hemodialysis. Case presentation A 70-year-old man undergoing hemodialysis was admitted because of skin purpura, abdominal pain, diarrhea, and lower back pain. We suspected him as IgA vasculitis based on the clinical features and skin biopsy findings. Transesophageal echocardiography revealed infective endocarditis, which predisposed him to IgA vasculitis. He was treated with antibiotics and low-dose corticosteroids, which led to resolution of vasculitis. Conclusions This is the first case of IgA vasculitis triggered by infective endocarditis in a patient undergoing hemodialysis. Patients undergoing hemodialysis are at a high risk of infection because of immune dysfunction and frequent venipuncture. The incidence of infective endocarditis associated with IgA vasculitis is very low, but it has been repeatedly reported. Therefore, it is necessary to consider infective endocarditis in patients with clinical features that indicate IgA vasculitis.


2019 ◽  
Vol 2019 ◽  
pp. 1-10 ◽  
Author(s):  
Jisup Kim ◽  
Sung-Eun Choi ◽  
Keum Hwa Lee ◽  
Hyeon Joo Jeong ◽  
Jae Il Shin ◽  
...  

Henoch-Schönlein purpura (HSP) is the most common systemic vasculitis in children, and renal involvement (HSP nephritis, HSPN) is a severe manifestation. HSPN is histologically classified by the International Study of Kidney Disease in Children (ISKDC) based on mesangial hypercellularity and the extent of glomerular crescents. Macrophages, categorized as M1 or M2, frequently infiltrate in various glomerular and tubulointerstitial diseases and infiltration of specific subtypes is associated with disease progression. Therefore, to identify whether infiltration of M1 or M2 macrophages has clinical significance, we quantified the subtypes of macrophages in 49 HSPN specimens and correlated the counts with histologic features and clinical parameters. Higher tubulointerstitial M2 counts were associated with chronic renal failure (CRF), ISKDC classes III-IV, and crescents (P<0.001, 0.002, 0.001). Glomerular M2 counts were significantly related to ISKDC classes III-IV and crescents (area under curve, AUC 0.804, 0.833). Tubulointerstitial M2 counts were associated with CRF, ISKDC classes III-IV, and crescents (AUC 0.872, 0.778, 0.830). Tubulointerstitial M2 counts also revealed higher AUC than tubulointerstitial M1 counts for CRF (P=0.036) and ISKDC classes III-IV (P=0.047). Glomerular M2 counts revealed higher AUC than glomerular M1 counts for ISKDC classes III–IV (P=0.024). Tubulointerstitial M2 counts were the most powerful parameter for CRF (AUC 0.872) and revealed even higher AUC than ISKDC classification (AUC 0.716) with borderline significance (P=0.086) for CRF. In summary, tubulointerstitial M2 counts were a superior parameter to tubulointerstitial M1 counts and even to ISKDC classification indicating the presence of CRF.


2020 ◽  
Vol 13 ◽  
pp. 117954412096737
Author(s):  
Vadood Javadi Parvaneh ◽  
Arezoo Shirzani ◽  
Khosro Rahmani ◽  
Reza Shiari

Background: Granulomatosis with polyangiitis (GPA) is a systemic vasculitis of the upper and lower respiratory tract along with glomerulonephritis and is very rare in childhood. Its renal manifestations similarity with IgA vasculitis can be misleading. Case presentation: Herein, we report a 12-years-old girl with the clinical picture of IgA vasculitis and renal involvement at the time of presentation, over time, elevated cytoplasmic Anti-neutrophil Cytoplasmic Antibody (C-ANCA) and tissue biopsy confirmed GPA. Conclusion: In the case of a patient with an unusual presentation of IgA vasculitis, to some degree of suspicion, the GPA should be considered. Also, in approach to non-thrombocytopenic palpable petechia and purpura a wide range of differential diagnosis such as infections, ANCA associated vasculitis, and secondary vasculitis should be considered. Therefore, 2 effective method of GPA diagnosis, the high titer of C-ANCA test and tissue biopsy, should be considered simultaneously.


Hematology ◽  
2005 ◽  
Vol 2005 (1) ◽  
pp. 368-372 ◽  
Author(s):  
Enrica Morra

Abstract Mixed cryoglobulinemia (MC) type II is a disorder characterized by circulating cold-precipitable immune complexes composed of polyclonal immunoglobulin IgG and monoclonal IgM rheumatoid factor (RF). The systemic vasculitis which characterizes the disease is caused by the deposition of immune complexes on the walls of small vessels, and by the subsequent activation of the complement cascade. MC may be asymptomatic or lead to clinical manifestations characterized by a typical triad—purpura, arthralgia, and weakness—and in some cases by a more serious vasculitis with neurologic and renal involvement. In the vast majority (more than 90%) of patients, MC is associated with hepatitis C virus (HCV) infection, which is considered the triggering factor of the disease. The association between cryoglobulinemia and HCV infection, and the possible evolution to B-cell lymphomas usually after long-term follow-up, suggest the role of HCV infection both in the pathogenesis of MC and in lymphomagenesis. In fact, the virus chronically stimulates the polyclonal proliferation of B cells from which a monoclonal population may emerge. This paper also reviews the treatment strategies for MC syndrome, emphasizing the issue of the eradication of HCV, and the clinical and biological activity of ritux-imab for selective B-cell control.


2021 ◽  
Vol 58 ◽  
pp. eUJ3780-eUJ3780
Author(s):  
Vergílio Pereira Carvalho ◽  

mmunoglobulin A vasculitis (IgAV) is known to be a systemic vasculitis of small vessels. Although is possible that this condition sets up in any age group, the epidemiological profile of this disease mostly affects the pediatric population between 2 and 10 years of age. In this sense, this descriptive article aims to report the rare case of an adult patient who evolved with IgAV, presented in a cutaneous form, and treated with corticosteroids. For that, after the patient's free and informed consent, the study was approved by the Research Ethics Committee of the University of Rio Verde (UniRV, Goiás, Brazil), whose Certificate of Presentation for Ethical Consideration is 36621920.0.0000.507. Therefore, it was found that the patient evolved with clinical compatible with IgAV of probable cutaneous involvement due to lesions in the form of purpura and renal involvement, presenting a good therapeutic response with corticosteroid therapy at non-immunosuppressive doses and after 1 year and 3 months of follow-up, there was remission of the clinicalpicture without new episodes.There are some reports in the literature about the good therapeutic response of this clinical with corticosteroid therapy at non-immunosuppressive doses, however, clinical trials are needed.


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