Renal involvement in Mediterranean spotted fever – clinical and histopathological data

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.

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

Journal of Clinical Medicine ◽

10.3390/jcm9072131 ◽

2020 ◽

Vol 9 (7) ◽

pp. 2131 ◽

Cited By ~ 2

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.

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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

BMC Nephrology ◽

10.1186/s12882-021-02495-8 ◽

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.

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Rickettsiosis

10.1093/med/9780199592548.003.0193 ◽

2015 ◽

Author(s):

Emmanuel A. Burdmann ◽

Vivekanad Jha

Keyword(s):

Serum Creatinine ◽

Spotted Fever ◽

Renal Involvement ◽

Adverse Outcomes ◽

Rickettsia Conorii ◽

Mediterranean Spotted Fever ◽

Spotted Fever Group ◽

Rocky Mountain Spotted Fever ◽

Murine Typhus ◽

Epidemic Typhus

Rickettsiae are obligate intracellular bacteria transmitted by arthropods to a vertebrate host. Clinically relevant rickettsioses have a similar clinical pattern, manifesting as an acute febrile disease accompanied by headache, articular and muscle pain, and malaise.Epidemic typhus is a worldwide distributed disease caused by the Rickettsia prowazekii, with a human louse as a vector. Data on epidemic typhus-related renal injury is extremely scarce.Murine typhus is caused by the Rickettsia typhi and has a rodent flea as the vector. It is one of the most frequent rickettsioses, and is usually a self-limited febrile illness. Proteinuria, haematuria, elevations in serum creatinine (SCr) and/or blood urea nitrogen (BUN) and AKI have been reported. The real frequency of renal involvement in murine typhus is unknown. Renal abnormalities recover after the infectious disease resolution.Scrub typhus, caused by the Orientia tsutsugamushi, has the Leptotrombidium mite larva as vector. It is endemic in the Tsutsugamushi triangle delimited by Japan, Australia, India, and Siberia. It can manifest either as a self-limiting disease or as a severe, life-threatening multiorgan illness. Early administration of adequate antibiotics is essential to prevent adverse outcomes. Proteinuria, haematuria, and acute kidney injury (AKI) are frequent.Tick-borne rickettsioses are caused by bacteria from the spotted fever group and have ticks as vectors. Rocky Mountain spotted fever (RMSF) is caused by Rickettsia rickettsii. It is the most severe of the spotted fever rickettsial diseases, causing significant morbidity and lethality. RMSF occurs in North, Central, and South America. Renal impairment is frequent in severe forms of RMSF. Mediterranean spotted fever is caused by Rickettsia conorii, and is endemic in the Mediterranean area. It is usually a benign disease, but may have a severe course, clinically similar to RMSF. Haematuria, proteinuria, increased serum creatinine, and AKI may occur. Japanese spotted fever is caused by Rickettsia japonica. Lethal cases are reported yearly and AKI has occurred in the context of multiple organ failure.

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Pathology of Antineutrophil Cytoplasmic Antibody–Associated Pulmonary and Renal Disease

Archives of Pathology & Laboratory Medicine ◽

10.5858/arpa.2016-0098-ra ◽

2017 ◽

Vol 141 (2) ◽

pp. 223-231 ◽

Cited By ~ 2

Author(s):

Maxwell L. Smith

Keyword(s):

Differential Diagnosis ◽

Renal Disease ◽

Clinical Laboratory ◽

Tissue Injury ◽

Renal Involvement ◽

Antineutrophil Cytoplasmic Antibody ◽

Small Vessel Vasculitis ◽

Antibody Testing ◽

Anca Associated Vasculitis ◽

Histopathologic Diagnosis

Context.—Antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis commonly presents with pulmonary and renal involvement that may present diagnostic challenges. Objectives.—To highlight the updates in the classification of small vessel vasculitis, present the patterns of pulmonary and renal pathology in which ANCA-associated vasculitis is included in the differential diagnosis, analyze the screening and specific antineutrophil cytoplasmic antibody testing methods in the clinical laboratory, compare and contrast the 3 major ANCA-associated vasculitis diseases, and review the pathophysiologic mechanisms of tissue injury in this setting. Data Sources.—Data are derived from published literature and clinical experience. Conclusions.—Although rare, ANCA-associated vasculitis diseases are often considered in the differential diagnosis of many pathologic patterns of pulmonary and renal disease. Histopathologic diagnosis of specific entities in this context nearly always requires correlation of the pathology with clinical and serologic data.

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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.

10.21203/rs.3.rs-308105/v1 ◽

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.

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Clinical Features and Outcomes of Immunoglobulin G4-related Disease

10.21203/rs.3.rs-243747/v1 ◽

2021 ◽

Author(s):

Sua Lee ◽

Chul Woo Yang

Keyword(s):

Clinical Features ◽

Clinical Laboratory ◽

Renal Involvement ◽

Multiple Organ ◽

Organ Involvement ◽

Response To Treatment ◽

Clinical Feature ◽

Immunoglobulin G4 ◽

Related Disease ◽

Kidney Involvement

Abstract Immunoglobulin G4-related disease (IgG4-RD) is a newly recognized disease, and a few epidemiologic studies about this disorder have been published. This research aimed to describe the clinical, laboratory, and histopathological features and outcomes of IgG4-RD. Ninety-four patients who satisfied the comprehensive diagnostic criteria on IgG4-RD were included in this study. Fifty-eight (61.7%) were men. The mean age was 54.8 years, and the median follow-up duration was 32.9 months. The clinical feature between single and multiple organ involvement and with or without kidney involvement groups were evaluated based on symptoms and laboratory findings. The clinical outcome was assessed according to treatment strategies and response. Of 94 patients, 56 (59.6%) had multiple organs involvement. Patients with multiple organ involvement had higher serum IgG and IgG4 levels than those with single organ involvement. Those with IgG4-related kidney disease (IgG4-RKD) had worse renal function, and the incidence of peripheral blood eosinophilia and hypocomplementemia was higher in patients with renal involvement than in those without. Glucocorticoids-based therapy was most commonly used. (79.8%). Thirty-nine (41.5%) achieved complete remission. Eighteen (19.1%) relapsed after response to treatment. IgG4-RD had different clinical features and outcomes. The number of organ involvement and kidney involvement were not associated with clinical outcomes.

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Mediterranean Spotted Fever: Clinical, Laboratory and Epidemiological Features of 199 Cases

American Journal of Tropical Medicine and Hygiene ◽

10.4269/ajtmh.1986.35.845 ◽

1986 ◽

Vol 35 (4) ◽

pp. 845-850 ◽

Cited By ~ 118

Author(s):

Didier Raoult ◽

Hervé Gallais ◽

Paul Casanova ◽

André Chagnon ◽

Hervé Chaudet ◽

...

Keyword(s):

Clinical Laboratory ◽

Spotted Fever ◽

Mediterranean Spotted Fever ◽

Epidemiological Features

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Systemic Lupus Erythematosus with and without Anti-dsDNA Antibodies: Analysis from a Large Monocentric Cohort

Mediators of Inflammation ◽

10.1155/2015/328078 ◽

2015 ◽

Vol 2015 ◽

pp. 1-6 ◽

Cited By ~ 3

Author(s):

Conti Fabrizio ◽

Ceccarelli Fulvia ◽

Perricone Carlo ◽

Massaro Laura ◽

Marocchi Elisa ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Disease Activity ◽

Lupus Erythematosus ◽

Clinical Laboratory ◽

Renal Involvement ◽

Serum Levels ◽

Activity Measurement ◽

Systemic Lupus ◽

Before And After ◽

Dsdna Antibodies

Objectives. The anti-dsDNA antibodies are a marker for Systemic Lupus Erythematosus (SLE) and 70–98% of patients test positive. We evaluated the demographic, clinical, laboratory, and therapeutical features of a monocentric SLE cohort according to the anti-dsDNA status.Methods. We identified three groups: anti-dsDNA + (persistent positivity); anti-dsDNA ± (initial positivity and subsequent negativity during disease course); anti-dsDNA − (persistent negativity). Disease activity was assessed by the European Consensus Lupus Activity Measurement (ECLAM).Results. We evaluated 393 patients (anti-dsDNA +: 62.3%; anti-dsDNA ±: 13.3%; anti-dsDNA −: 24.4%). The renal involvement was significantly more frequent in anti-dsDNA + (30.2%), compared with anti-dsDNA ± and anti-dsDNA − (21.1% and 18.7%, resp.;P=0.001). Serositis resulted significantly more frequent in anti-dsDNA − (82.3%) compared to anti-dsDNA + and anti-dsDNA ± (20.8% and 13.4%, resp.;P<0.0001). The reduction of C4 serum levels was identified significantly more frequently in anti-dsDNA + and anti-dsDNA ± (40.0% and 44.2%, resp.) compared with anti-dsDNA − (21.8%,P=0.005). We did not identify significant differences in the mean ECLAM values before and after modification of anti-dsDNA status (P=0.7).Conclusion. Anti-dsDNA status influences the clinical and immunological features of SLE patients. Nonetheless, it does not appear to affect disease activity.

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