scholarly journals Assessment of the correlation of commonly used laboratory tests with clinical activity, renal involvement and treatment of systemic small-vessel vasculitis with the presence of ANCA antibodies

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Magdalena Mosakowska ◽  
Dorota Brodowska Kania ◽  
Katarzyna Szamotulska ◽  
Aleksandra Rymarz ◽  
Stanisław Niemczyk
Keyword(s):  
Peripheral Blood ◽  
Laboratory Tests ◽  
Renal Involvement ◽  
Infectious Complications ◽  
Microscopic Hematuria ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
Clinical Activity ◽  
Kidney Involvement ◽  
D Dimer

Abstract Background The aim of the study was to assess the correlation of commonly used laboratory tests with clinical activity, degree of kidney involvement and treatment of systemic small-vessel vasculitis with the presence of ANCA antibodies. Methods The study included 28 patients with active AAV (BVAS ≥ 3). The following tests were performed: MPO-ANCA, PR3-ANCA, peripheral blood count, ESR, CRP, procalcitonin, creatinine, GFR, urea, albumin, fibrinogen, d-dimer, components of the C3 and C4 complement systems, urinalysis with sediment evaluation and diurnal proteinuria. The assessments were conducted twice: at study entry (A0) and after 6 months (A6) (BVAS = 0). Results At the time of inclusion in the study, the mean creatinine concentration was 3.39 mg/dl (GFR 33.17 ml/min/1.73 m²), after achieving remission in 11 patients (39.3 %) GFR remained below 30 ml/min/1.73 m², 4 patients (14.3 %) continued renal replacement therapy, and 3 patients (10.7 %) with advanced renal failure died. Microscopic hematuria occurred in 80.9 % of the studied population, withdrew in most patients, strongly correlated with renal involvement p < 0.001 and was not related to disease severity p = 0.147. CRP, ESR, fibrinogen, d-dimer, albumin and hemoglobin in the peripheral blood showed a strong correlation with the clinical activity of AAV and well identified severe patients. High procalcitonin concentrations correlated with a severe form of the disease, pulmonary involvement with respiratory failure and alveolar hemorrhage (mean 3.41 ng/ml, median 0.91 ng/ml, SD 7.62, p = 0.000), and were associated with the occurrence of infectious complications and the need to administer antibiotic therapy. ANCA antibodies were useful in the evaluation of patients with AAV, the amount of antibodies did not correlate with the severity of vasculitis (p = 0.685) and the results in many patients did not match the expected assumptions. Conclusions CRP, ESR, fibrinogen, d-dimers, albumin and hemoglobin in the peripheral blood correlate well with the activity of vasculitis and identify severe patients. The resolution of microscopic hematuria suggests remission of the disease in the renal area. Procalcitonin may be slightly increased in patients with active AAV without infection, high concentrations are strongly associated with infectious complications. ANCA antibodies should always be interpreted in the context of the observed clinical symptoms.

scholarly journals Assessment of the Correlation of Commonly used Laboratory Tests with Clinical Activity, Renal Involvement and Treatment of Systemic Small-vessel Vasculitis with the Presence of ANCA Antibodies. 

2021 ◽  
Author(s):  
Magdalena Mosakowska ◽  
Dorota Brodowska-Kania ◽  
Katarzyna Szamotulska ◽  
Aleksandra Rymarz ◽  
Stanisław Niemczyk
Keyword(s):  
Peripheral Blood ◽  
Laboratory Tests ◽  
Clinical Symptoms ◽  
Renal Involvement ◽  
Infectious Complications ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
Clinical Activity ◽  
Kidney Involvement ◽  
D Dimer

Abstract The aim of the study was to assess the correlation of commonly used laboratory tests with clinical activity, degree of kidney involvement and treatment of systemic small-vessel vasculitis with the presence of ANCA antibodies.Methods.The study included 28 patients with active AAV (BVAS ≥ 3). The following tests were performed: MPO-ANCA, PR3-ANCA, peripheral blood count, ESR, CRP, procalcitonin, creatinine, GFR, urea, albumin, fibrinogen, d-dimer, components of the C3 and C4 complement systems, urinalysis with sediment evaluation and diurnal proteinuria. The assessments were conducted twice: at study entry (A0) and after 6 months (A6) (BVAS = 0).ResultsAt the time of inclusion in the study, the mean creatinine concentration was 3.39 mg/dl (GFR 33.17 ml/min/1.73m²), after achieving remission in 11 patients (39.3%) GFR remained below 30 ml/min/1.73m² , 4 patients (14.3%) continued renal replacement therapy, and 3 patients (10.7%) with advanced renal failure died. Microhematuria occurred in 80.9% of the studied population, withdrew in most patients, strongly correlated with renal involvement p<0.001 and was not related to disease severity p=0.147. CRP, ESR, fibrinogen, d-dimer, albumin and hemoglobin in the peripheral blood showed a strong correlation with the clinical activity of AAV and well identified severe patients. High procalcitonin concentrations correlated with a severe form of the disease, pulmonary involvement with respiratory failure and alveolar hemorrhage (mean 3.41 ng/ml, median 0.91 ng/ml, SD 7.62, p = 0.000), and were associated with the occurrence of infectious complications and the need to administer antibiotic therapy. ANCA antibodies were useful in the evaluation of patients with AAV, the amount of antibodies did not correlate with the severity of vasculitis (p=0.685) and the results in many patients did not match the expected assumptions. ConclusionsCRP, ESR, fibrinogen, d-dimers, albumin and hemoglobin in the peripheral blood correlate well with the activity of vasculitis and identify severe patients. The resolution of microhematuria suggests remission of the disease in the renal area. Procalcitonin may be slightly increased in patients with active AAV without infection, high concentrations are strongly associated with infectious complications. ANCA antibodies should always be interpreted in the context of the observed clinical symptoms.

scholarly journals Kidney Involvement in Hypocomplementemic Urticarial Vasculitis Syndrome—A Case-Based Review

2020 ◽  
Vol 9 (7) ◽  
pp. 2131 ◽  
Author(s):  
Oana Ion ◽  
Bogdan Obrișcă ◽  
Gener Ismail ◽  
Bogdan Sorohan ◽  
Sonia Bălănică ◽  
...  
Keyword(s):  
Current Knowledge ◽  
Renal Involvement ◽  
Immunosuppressive Treatment ◽  
Small Vessel Vasculitis ◽  
Extensive Literature ◽  
Urticarial Vasculitis ◽  
Pattern Of Injury ◽  
Kidney Involvement ◽  
Hypocomplementemic Urticarial Vasculitis Syndrome ◽  
Hypocomplementemic Urticarial Vasculitis

Hypocomplementemic urticarial vasculitis syndrome (HUVS), or McDuffie syndrome, is a rare small vessel vasculitis associated with urticaria, hypocomplementemia and positivity of anti-C1q antibodies. In rare cases, HUVS can manifest as an immune-complex mediated glomerulonephritis with a membranoproliferative pattern of injury. Due to the rarity of this disorder, little is known about the clinical manifestation, pathogenesis, treatment response and outcome of such patients. We describe here three cases of HUVS with severe renal involvement. These patients had a rapidly progressive form of glomerulonephritis with severe nephrotic syndrome against a background of a membranoproliferative pattern of glomerular injury with extensive crescent formation. Therefore, these patients required aggressive induction and maintenance immunosuppressive therapy, with a clinical and renal response in two patients, while the third patient progressed to end-stage renal disease. Because of the rarity of this condition, there are few data regarding the clinical presentation, pathology and outcome of such patients. Accordingly, we provide an extensive literature review of cases reported from 1976 until 2020 and place them in the context of the current knowledge of HUVS pathogenesis. We identified 60 patients with HUVS and renal involvement that had adequate clinical data reported, out of which 52 patients underwent a percutaneous kidney biopsy. The most frequent renal manifestation was hematuria associated with proteinuria (70% of patients), while one third had abnormal kidney function on presentation (estimated glomerular filtration (GFR) below 60 mL/min/1.73 m2). The most frequent glomerular pattern of injury was membranoproliferative (35%), followed by mesangioproliferative (21%) and membranous (19%). Similar to other systemic vasculitis, renal involvement carries a poorer prognosis, but the outcome can be improved by aggressive immunosuppressive treatment.

scholarly journals Renal involvement in Mediterranean spotted fever – clinical and histopathological data

2021 ◽  
Author(s):  
Ivan Baltadzhiev ◽  
Nedialka Popivanova ◽  
Atanas Baltadjiev
Keyword(s):  
Renal Impairment ◽  
Clinical Laboratory ◽  
Spotted Fever ◽  
Renal Involvement ◽  
Small Vessel Vasculitis ◽  
Mediterranean Spotted Fever ◽  
Rickettsial Infection ◽  
Tubular Necrosis ◽  
Kidney Involvement ◽  
Imported Cases

Background: Mediterranean spotted fever (MSF) is a tick-borne rickettsial infection endemic to the Mediterranean coastline countries. As a result of growing tourism imported cases have been registered in many non-endemic countries and regions. Objective: We present clinical laboratory parameters and histopathological data on renal impairment in patients with MSF. The study meets our goal of identifying kidney involvement and detecting renal damage in people with MSF. Subjects and Methods: 350 patients with MSF with a diagnosis confirmed by immunofluorescence analysis were tested for serum urea, creatinine and albumin. Fifty five patients with malignant form of MSF were divided into two groups: 19 fatalities and 36 survivors. The percentage of patients with acute renal failure (ARF) was compared in both groups. Results: Subjects with elevated urea and creatinine levels increased from 5.21% and 3.47% in mild to 48.78% and 29.26% in severe MSF, respectively. Loss of serum albumin also increased from mild to severe MSF. Renal impairment comprised 60% of the cohort of 55 patients with malignant MSF: 89.4% in the group of deaths, and almost twice less in the survivors. ARF developed in 84.2% of fatal cases and was more than two times less in survivors. The postmortem performed light microscopy of renal samples of 9 fatal cases revealed perivascular mononuclear inflammatory infiltrates, vasculitis with fibrinoid necrosis, acute tubular necrosis, interstitial edema, hemorrhage and thrombosis. Conclusion: Renal pathology associated with MSF rickettsial infection consists of systemic small vessel vasculitis and vascular injury leading to ARF in the most severe cases.

scholarly journals Renal involvement in granulomatosis with polyangiitis

2020 ◽  
Vol 8 (6) ◽  
pp. 158-159
Author(s):  
Aya Fraj ◽  
Olfa Berriche ◽  
Sondes Arfa ◽  
Jihen Chelli ◽  
Wafa Bel Abed ◽  
...  
Keyword(s):  
Renal Failure ◽  
Respiratory Tract ◽  
Granulomatosis With Polyangiitis ◽  
Renal Involvement ◽  
Rapidly Progressive Glomerulonephritis ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
Upper Respiratory Tract ◽  
Terminal Renal Failure ◽  
Upper Respiratory

Granulomatosis with polyangiitis (GPA) is defined by the presence of small vessel vasculitis that affects the upper respiratory tract, lungs and kidneys. Renal involvement is frequent. The disease varies from asymptomatic to fulminant rapidly progressive glomerulonephritis forms, leading to terminal renal failure in days.

scholarly journals Diagnosing of Small Vessel Vasculitis Might be a Challenge – A Rare Case Report

2020 ◽  
Vol 49 (2) ◽  
pp. 41-43
Author(s):  
Md Abu Shahin ◽  
Mohammad Mamun Khan ◽  
Swikriti Shrestha ◽  
Syed Jamil Abdal ◽  
Md Ariful Islam ◽  
...  
Keyword(s):  
Renal Involvement ◽  
Upper Airway ◽  
Multiple Organ ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
Glucocorticoid Treatment ◽  
Neurologic Involvement ◽  
Rare Case Report ◽  
Life Threatening ◽  
Single Organ

The term vasculitis refers to the inflammation of vessel walls. It may range in severity from a self-limited disorder in one single organ to a life-threatening disease due to multiple organ failure. Most patients with small vessel vasculitis present with constitutional symptoms like fever, malaise, weakness, fatigue and weight loss. To diagnose small vessel vasculitis, serology like ANCA, serum cryoglobulin and biopsy play an important role. Despite the serology and biopsy, diagnosing small vessel vasculitis occasionally remains challenging in resource constraint countries. Here we are reporting a case of a 26-year- old female who presented with purpura and neuropathy. The patient lacks clinical features like constitutional symptoms, renal involvement, upper airway involvement and her ANCA was negative. Depending on biopsy finding and skin and neurologic involvement, we diagnosed her as a case of small vessel vasculitis (unclassified). The patient improved with IV methylprednisolone followed by oral glucocorticoid treatment along with methotrexate. Although small vessel vasculitis has some typical features, diagnosis may often remain challenging even after biopsy. Bangladesh Med J. 2020 May; 49(2) : 41-43

scholarly journals Henoch-Scholein Purpura (HSP): A Clinico-etiological Study

2016 ◽  
Vol 12 (1) ◽  
pp. 38-33
Author(s):  
SN Kareem ◽  
N Riyaz
Keyword(s):  
Age Of Onset ◽  
Renal Involvement ◽  
Gastrointestinal Symptoms ◽  
Occult Blood ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
Joint Involvement ◽  
Upper Respiratory Tract ◽  
Palpable Purpura ◽  
American College Of Rheumatology

Background: Henoch–Schönlein (HSP) is a small vessel vasculitis affecting skin and internal organs.  Objectives: To determine the age group affected, etiological factors, clinical features, complications and association with other diseases. Methods: The study included 50 patients over a period of 3 years selected on the modified criteria by American College of Rheumatology. Clinical assessment was done and investigated for any focus of hidden infections especially Dental and ENT by respective departments. Associated conditions, complications and recurrence were also observed. Results: All patients had palpable purpura (pruritic in 58% and with hemorrhagic bullae in 6%) with upper respiratory tract prodrome (44%), joint involvement (94%) and gastrointestinal symptoms (90%). Precipitating factors were sore throat (74%), caries tooth (44%), drugs (30%) and food (16%) in varying combinations. On investigation 56% had raised ESR and 22% were with abnormal peripheral smears. All biopsies showed leucocytoclastic vasculitis. Occult blood in stool was detected in 10% and 2% showed Streptococci in throat swab culture. Renal involvement was 54% (RBC and albumin-42%, RBC alone-12%). One patient had intestinal perforation. Bleeding time, Clotting time, Platelet count, Cryoglobulin, ANA, Rheumatoid factor, HBs Ag, VDRL and X-ray chest were within normal limits. LE cell test was negative. Follow up study showed recurrence in 98%. Conclusion: HSP is a small vessel vasculitis in response to any unknown foreign or endogenous antigen. The age of onset varied from below 7 to 58 years, with most of them below 20. The most important clinical presentation was palpable purpura in lower extremities. GIT and joint involvement were more. Renal involvement found to be the most common complication. DOI: http://dx.doi.org/10.3126/njdvl.v12i1.10593 Nepal Journal of Dermatology, Venereology & Leprology Vol.12(1) 2014 pp.28-33 

Validating identification of patients with small vessel vasculitis, with or without renal involvement, using administrative healthcare records

2017 ◽  
Vol 87 (03) ◽  
pp. 159-162
Author(s):  
Michelle M. O’Shaughnessy ◽  
XingXing S. Cheng ◽  
Maria E. Montez- Rath ◽  
Richard A. Lafayette ◽  
Wolfgang C. Winkelmayer
Keyword(s):  
Renal Involvement ◽  
Small Vessel ◽  
Small Vessel Vasculitis
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