scholarly journals Methimazole-Induced ANCA Vasculitis: A Case Report

Diagnostics ◽  
2021 ◽  
Vol 11 (9) ◽  
pp. 1580
Author(s):  
Precil Diego Miranda de Menezes Neves ◽  
Lucas Braga Mota ◽  
Cristiane Bitencourt Dias ◽  
Luis Yu ◽  
Viktoria Woronik ◽  
...  
Keyword(s):  
Urine Analysis ◽  
Interstitial Fibrosis ◽  
White Cell Count ◽  
Rapidly Progressive Glomerulonephritis ◽  
Alveolar Hemorrhage ◽  
Fibrinoid Necrosis ◽  
High Power Field ◽  
Serological Tests ◽  
Anca Vasculitis ◽  
Bronchial Washing

Rapidly progressive glomerulonephritis (RPGN) is a syndrome which presents rapid loss of renal function. Vasculitis represents one of the major causes, often related to anti-neutrophil cytoplasmic antibodies (ANCA). Herein, we report a case of methimazole-induced ANCA-associated vasculitis. A 35-year-old woman complained of weight loss and fatigue for 2 weeks and attended the emergency room with alveolar hemorrhage. She had been diagnosed with Graves’ disease and had been taking methimazole in the past 6 months. Her physical examination showed pulmonary wheezing, hypertension and signs of respiratory failure. Laboratory tests revealed urea 72 mg/dL, creatinine 2.65 mg/dL (eGFR CKD-EPI: 20 mL/min/1.73 m2), urine analysis with >100 red blood cells per high-power field, 24 h-proteinuria: 1.3 g, hemoglobin 6.6 g/dL, white-cell count 7700/mm3, platelets 238,000/mm3, complement within the normal range, negative viral serological tests and ANCA positive 1:80 myeloperoxidase pattern. Chest tomography showed bilateral and diffuse ground-glass opacities, and bronchial washing confirming alveolar hemorrhage. A renal biopsy using light microscopy identified 27 glomeruli (11 with cellular crescentic lesions), focal disruption in glomerular basement membrane and fibrinoid necrosis areas, tubulitis and mild interstitial fibrosis. Immunofluorescence microscopy showed IgG +2/+3, C3 +3/+3 and Fibrinogen +3/+3 in fibrinoid necrosis sites. She was subsequently diagnosed with crescentic pauci-immune glomerulonephritis, mixed class, in the setting of a methimazole-induced ANCA vasculitis. The patient was treated with methimazole withdrawal and immunosuppressed with steroids and cyclophosphamide. Four years after the initial diagnosis, she is currently being treated with azathioprine, and her exams show creatinine 1.30 mg/dL (eGFR CKD-EPI: 52 mL/min/1.73 m2) and negative p-ANCA.

Antiglomerular Basement Membrane Disease

2018 ◽  
Vol 39 (04) ◽  
pp. 494-503 ◽  
Author(s):  
Charles Pusey ◽  
Stephen McAdoo
Keyword(s):  
Basement Membrane ◽  
Rapidly Progressive Glomerulonephritis ◽  
Basement Membranes ◽  
Alveolar Hemorrhage ◽  
Type Iv ◽  
Maintenance Immunosuppression ◽  
Life Threatening ◽  
Renal Prognosis ◽  
Function Testing

AbstractAntiglomerular basement membrane (anti-GBM) disease is a rare but life-threatening autoimmune vasculitis that is characterized by the development of pathogenic autoantibodies to type IV collagen antigens expressed in the glomerular and alveolar basement membranes. Once deposited in tissue, these autoantibodies incite a local capillaritis which manifests as rapidly progressive glomerulonephritis (GN) in 80 to 90% of patients, and with concurrent alveolar hemorrhage in ∼50%. A small proportion of cases may present with pulmonary disease in isolation. Serological testing for anti-GBM antibodies may facilitate rapid diagnosis, though renal biopsy is often required to confirm the presence of necrotizing or crescentic GN and linear deposition of autoantibody on the glomerular basement membrane. Alveolar hemorrhage may be evident clinically, or detected on imaging, pulmonary function testing, or bronchoscopy. Prompt treatment with plasmapheresis, cyclophosphamide, and steroids is usually indicated to remove pathogenic autoantibodies, to prevent their ongoing production, and to ameliorate end-organ inflammation. Alveolar hemorrhage is usually responsive to this treatment, and long-term respiratory sequelae are uncommon. Renal prognosis is more variable, though with aggressive treatment, independent renal function is maintained at 1 year in more than 80% of patients not requiring renal replacement therapy at presentation. Relapse in uncommon in anti-GBM disease, unless there is a concomitant antineutrophil cytoplasm antibody (present in 30–40%), in which case maintenance immunosuppression is recommended.

scholarly journals Atypical Goodpasture’s disease: a clinical case report and literature review

2018 ◽  
Vol 90 (6) ◽  
pp. 130-136 ◽  
Author(s):  
M L Bulanova ◽  
D V Potapov ◽  
N M Bulanov ◽  
L V Lysenko(Kozlovskaya)
Keyword(s):  
Basement Membrane ◽  
Renal Involvement ◽  
Rapidly Progressive Glomerulonephritis ◽  
Young Male ◽  
Alveolar Hemorrhage ◽  
Disease Course ◽  
Small Vessels ◽  
Goodpasture's Disease ◽  
Pulmonary Renal Syndrome ◽  
Goodpasture’S Disease

Goodpasture’s disease (anti-GBM disease) is a rare small vessels vasculitis characterized by the presence of autoantibodies directed against the glomerular basement membrane (GBM) and alveolar basement membrane. Common feature of anti-GBM disease is a combination of rapidly progressive glomerulonephritis and alveolar hemorrhage (pulmonary-renal syndrome). We present a case of atypical disease course in a young male patient who developed alveolar hemorrhage without renal failure. The only symptom of renal involvement was isolated hematuria. Plasmapheresis combined with immunosuppression (cyclophosphamide and corticosteroids) was effective. We present a review of state-of-art data on the pathogenesis and disease course of anti-GBM disease.

Benefits and Problems of Routine Laboratory Investigations in Adult Psychiatric Admissions

1989 ◽  
Vol 155 (1) ◽  
pp. 65-72 ◽  
Author(s):  
Anthony J. White ◽  
Brian Barraclough
Keyword(s):  
Mental Illness ◽  
Cell Count ◽  
Urine Analysis ◽  
White Cell Count ◽  
District General Hospital ◽  
Physical Illness ◽  
White Cell ◽  
Psychiatric Admissions ◽  
Laboratory Investigations ◽  
Test Use

A survey of laboratory investigations in the management of 1007 non-geriatric admissions to a district general hospital adult psychiatric unit showed a wide variation in the number of tests performed, and in the abnormal proportion between test types. Despite a policy of screening on admission, 40% of admissions had no tests. Physical illness attributable to mental illness was common, but rarely serious and usually apparent clinically. Mental illness attributable to physical illness detected by laboratory tests was rare: five cases of thyroid disease in women with affective disorder, and two cases of chest infection with raised white cell count in sustained mania. The findings show that tests are frequently used in circumstances where the result is of no apparent value. Test use might be improved by restricting screening to thyroid function and laboratory urine analysis, each in women. There are particular indications for white cell count in sustained or unexplained relapse of mental illness, and for syphilis serology when disinhibition may have exposed the patient to infection.

Pancytopenia a Presentation of disseminated Histoplasmosis

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 4687-4687
Author(s):  
Sakeer Hussain
Keyword(s):  
Rheumatoid Arthritis ◽  
Bone Marrow ◽  
Histoplasma Capsulatum ◽  
Urine Analysis ◽  
Conflicts Of Interest ◽  
White Cell Count ◽  
Oral Prednisone ◽  
Mediastinal Lymphadenopathy ◽  
Fungal Culture ◽  
Disseminated Histoplasmosis

Abstract Abstract 4687 A 70 year old female with diabetes mellitus, rheumatoid arthritis with worsening fatigue, intermittent shaking chills and fever for 2 weeks. She had been on oral prednisone 5 mg daily and methotrexate 15 mg every week for her rheumatoid arthritis. Vital signs were stable and physical examination was significant for 3+ lower extremity edema and pallor. The CBC showed pancytopenia, white cell count of 2.5×109/L (4.3–10.8×109/L), hemoglobin was 9.2g/dl (11.0–15.0g/dl), and platelet counts were 93 × 109/L (130–400 × 109/L). SHe had mildly elevated total bilirubin 1.7mg/dl (0.0–1.2mg/dl), elevated alkaline phosphate 1212U/L (38–126 U/L) liver enzymes, SGPT 83U/L (0–40U/L), SGOT 126U/L (0–35U/L), and elevated blood sugar of 260mg/dl. Blood cultures remain negative for bacterial growth for multiple days. Her SPEP, ANA, HIV, CMV, and EBV serology were negative. CT scan of the chest, abdomen and pelvis were consistent with hilar, mediastinal lymphadenopathy and upper abdominal lymphadenopathy with splenomegaly. A bone marrow aspiration and biopsy was consistant with histoplasma capsulatum infection. Patient was treated with IV amphotericin B for 4 weeks. Histoplasma capsulatum was identified from fungal culture from the bone marrow aspirate. Retrospectively urine analysis for histoplasma antigen was positive by ELISA. Subsequently patient recieved itraconazole for 6 months. Cytopenia and symptoms improved and lymphadenopathy regressed. Most patoents who develop disseminated histoplasmosis are immunosuppressed It often mimic the presentation of malignancy like lymphoma or infection like tuberculosis and inflammatory process like sarcoidosis. Misdiagnosis can lead to inappropriate and harmful therapy with glucocorticoides and cytotoxic chemotherapy. Diagnosis requires high index of suspicion and familiarity with clinical presentation and appropriate diagnostic test. Disclosures: No relevant conflicts of interest to declare.

scholarly journals For fulminant rapidly progressive glomerulonephritis in a 17-year-old female patient with ANCA-associated vasculitis.

2018 ◽  
Vol 96 (2) ◽  
pp. 180-185
Author(s):  
Nadezhda B. Gordovskaya ◽  
E. M. Shilov ◽  
Yu. V. Korotchaeva ◽  
E. V. Stavrovskaya ◽  
S. V. Roshchupkin
Keyword(s):  
Renal Failure ◽  
Kidney Transplantation ◽  
Rapidly Progressive Glomerulonephritis ◽  
Proteinase 3 ◽  
Substitution Therapy ◽  
Anca Vasculitis ◽  
Terminal Renal Failure ◽  
Anca Associated Vasculitis ◽  
Morphological Picture ◽  
Systemic Manifestations

A case of rapidly progressive glomerulonephritis in a 17-year-old patient associated with antibodies against the cytoplasm of neutrophils (ANCA) vasculitis - ANCA-associated vasculitis is associated with antibodies to proteinase-3 and morphological picture extracapillar glomerulonephritis with sclerotic lesion of up to 80% of the glomeruli. The peculiarity of the case is the presence of morphologically confirmed when alloimmune rapidly progressive glomerulonephritis type III a pronounced glow-focal granular nature of immunoglobulin classes G and M on the basement membrane of capillaries. The appointment of immunosuppressive therapy led to a decrease in systemic manifestations of vasculitis, but there was a rapid increase in terminal renal failure, which required substitution therapy with hemodialysis. The possible mechanisms of the rapid-training course of the disease in the observed patient, prospects for kidney transplantation are discussed.

scholarly journals Rapidly progressive glomerulonephritis and acute kidney injury associated with cocaine use - Case report

2020 ◽  
Author(s):  
Paulo Vitor de Souza Pimentel ◽  
Hermany Capistrano Freitas ◽  
Marcos Diógenes Braga Leite ◽  
Rafael Siqueira Athayde Lima ◽  
Dulce Maria Sousa Barreto ◽  
...  
Keyword(s):  
Acute Kidney Injury ◽  
Renal Function ◽  
Wide Spectrum ◽  
Rapidly Progressive Glomerulonephritis ◽  
Kidney Injury ◽  
Percutaneous Biopsy ◽  
Pulse Therapy ◽  
Tubular Atrophy ◽  
Anca Vasculitis ◽  
Nephritic Syndrome

ABSTRACT A wide spectrum of renal complications can occur with acute and chronic use of cocaine. Most cases are related to rhabdomyolysis, but other mechanisms are malignant hypertension, renal ischemia, and rapidly progressive glomerulonephritis (RPGN) associated-ANCA vasculitis. In recent years, the use of cocaine adulterated with levamisole has been associated with ANCA vasculitis and pauci-immune RPGN. RPGN is clinically manifested as a nephritic syndrome with a rapid and progressive decline in renal function, and its histopathological finding is the presence of crescents in more than 50% of the glomeruli. We report a case of a 38-year-old man chronic user of cocaine, alcohol, and cigarettes who had red urine, oliguria, swollen legs and eyelids, as well as the uremic symptoms anorexia, emesis, and mental confusion. He was admitted with acute kidney injury and performed six hemodialysis sessions during the first 16 days of hospitalization and then was transferred to a tertiary hospital for diagnostic investigation. Tests of ANF (antinuclear factor), ANCA, anti-DNA, serology for hepatitis B, C, and HIV virus were negative. A renal percutaneous biopsy revealed crescentic glomerulonephritis with mild tubular atrophy. The patient underwent pulse therapy with methylprednisolone (for 3 days) and cyclophosphamide. Then he maintained daily prednisone and monthly intravenous cyclophosphamide and evolved with progressive improvement of renal function.

scholarly journals Churg-Strauss Syndrome Presenting with Diffuse Alveolar Hemorrhage and Rapidly Progressive Glomerulonephritis

2009 ◽  
Vol 48 (20) ◽  
pp. 1807-1811 ◽  
Author(s):  
Keishi Sugino ◽  
Naoshi Kikuchi ◽  
Yoko Muramatsu ◽  
Go Sano ◽  
Emiko Kusano ◽  
...  
Keyword(s):  
Rapidly Progressive Glomerulonephritis ◽  
Diffuse Alveolar Hemorrhage ◽  
Alveolar Hemorrhage ◽  
Churg Strauss Syndrome ◽  
Strauss Syndrome ◽  
Churg Strauss

scholarly journals FC 065PREDICTING OUTCOMES IN ANCA ASSOCIATED VASCULITIS: THE COMPLETE SCOTTISH EXPERIENCE

2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Dominic McGovern ◽  
Jennifer Lees ◽  
Dana Kidder ◽  
James Smith ◽  
Jamie Traynor ◽  
...  
Keyword(s):  
High Risk ◽  
Renal Biopsy ◽  
Interstitial Fibrosis ◽  
Low Risk ◽  
Risk Category ◽  
Proportional Hazard ◽  
Anca Vasculitis ◽  
Cox Proportional Hazard ◽  
High Risk Category ◽  
Risk Categories

Abstract Background and Aims Outcomes in ANCA vasculitis remain difficult to predict and therapeutic decision-making can be challenging. We aimed to establish if a renal risk score (RRS) could predict outcomes in this population. Method The Scottish Renal Biopsy Registry is a complete national dataset of all renal biopsies performed in Scotland. Those who had a first renal biopsy between 01/01/2014 and 31/12/2017 with evidence of ANCA vasculitis were included. Demographic data, treatment regimens, episodes of relapse and patient and kidney survival were recorded, retrospectively. The RRS was calculated using the system proposed by Brix et al (1). Each patient was categorised according to % of normal glomeruli (N0 >25%, N1 10 to 25%, N2 <10%), % of tubular atrophy/interstitial fibrosis (T0 ≤25%, T1 >25%) and eGFR (CKD-EPI) at time of biopsy (eGFR: G0 >15 mL/min/1.73 m2, G1 ≤15 mL/min/1.73 m2). Individual scores were summated and patients defined as low, medium or high risk. Cox proportional hazard models were created for survival to ESKD, relapse and death, stratified by risk category. Analyses were conducted using R statistical software. Results Two-hundred and forty-six patients with biopsy proven ANCA vasculitis were identified. Fifty percent (n=123), 46% (n=112) and 5% (n=11) were stratified as low, medium and high risk respectively. Fifty-two percent (n=129) were male and mean age at biopsy was 66.7±12.2 years. This was similar across the risk categories. Mean eGFR was lower in the high-risk category (High risk 8.6±6.1 ‘v’ Low risk 45.7±26.0 ml/min/1.73m2, p<0.001) and proteinuria was higher (High risk 405 (IQR 170-767) ‘v’ Low risk 81 (IQR 41-155) mg/mmol, p<0.001). Thirty-seven percent (n=91) were PR3 antigen positive, 2% (n=5) had dual positivity. In the high risk category, 8 (73%) were PR3 or dual positive. Eighteen (n=7%) patients experienced pulmonary haemorrhage; representation similar across all risk categories. Those categorised as medium or high risk were more likely to receive plasma exchange and/or haemodialysis at presentation (p<0.001) compared with the low risk category. Overall, 16% (n=40) of patients relapsed with a trend to higher risk of relapse in the low risk group (27% of these patients, p=0.05). Thirty seven (15%) patients developed ESKD. Cox proportional hazard model for development of ESKD (Figure 1) shows that those in high risk ‘v’ low risk category were more likely to reach ESKD (HR 124.8, 95% CI 26.4-590.3, p<0.001). Patient survival was similar between risk categories. Conclusion A simple RRS, using routinely reported data, in patients with renal biopsy proven ANCA vasculitis can help to predict development of ESKD. It may also be predictive of future relapse in those with a lower RRS, most likely explained by reduced irreversible damage in this group. The RRS could inform monitoring and treatment decisions. Whilst the numbers are small, a unique strength of this data is that it is based on a complete national dataset making it less susceptible to bias from regional variations in diagnostic and therapeutic practice.

scholarly journals Clinical and Histological Outcome Predictors in Renal Limited Pauci-Immune Crescentic Glomerulonephritis: A Single Centre Experience

2012 ◽  
Vol 25 (1) ◽  
pp. 287-292 ◽  
Author(s):  
A. Gigante ◽  
C. Salviani ◽  
K. Giannakakis ◽  
E. Rosato ◽  
B. Barbano ◽  
...  
Keyword(s):  
Serum Creatinine ◽  
Crescentic Glomerulonephritis ◽  
Alternative Pathway ◽  
Rapidly Progressive Glomerulonephritis ◽  
Renal Outcome ◽  
Fibrinoid Necrosis ◽  
Histological Features ◽  
Systemic Involvement ◽  
University Of Rome

Renal-limited vasculitis is a pauci-immune crescentic glomerulonephritis with no signs of systemic involvement, representing one of the most common causes of rapidly progressive glomerulonephritis. The study aims to examine clinical and histological features in twenty-four patients with RLV diagnosed by the Nephrology Department of Sapienza University of Rome, Italy, evaluating the role of these parameters in predicting renal survival. Patients details, clinical and histological features and outcomes were recorded at the time of renal biopsy and over a mean follow-up period of 36±6 months. In our study, serum creatinine at presentation was significantly higher in patients who had a poor outcome than in those who survived with independent renal function (6.3±2.47 mg/dl vs 2.84±2.01 mg/dl, P= 0.002). The presence of C3c was found in the area of glomerular fibrinoid necrosis and in small arteries and arterioles with fibrinoid necrosis in 17 patients (P= 0.018). In conclusion, serum creatinine at presentation and focal C3c depositions in areas of glomerular and arteriolar fibrinoid necrosis were the best determinants of poor renal outcome, maybe underlining the pathogenic role of alternative pathway activation of complement system but also demonstrating the focal distribution of necrotizing lesions.

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