scholarly journals A case of crescentic glomerulonephritis associated with relapsing polychondritis

2018 ◽  
Vol 8 (1) ◽  
pp. 62-64
Author(s):  
Bahman Bashardoust ◽  
Ahmad Sabzevari ◽  
Mojgan Asgari
Keyword(s):  
Renal Failure ◽  
Autoimmune Disease ◽  
Inflammatory Arthritis ◽  
Crescentic Glomerulonephritis ◽  
Cartilage Damage ◽  
Renal Involvement ◽  
Relapsing Polychondritis ◽  
Rare Presentation ◽  
Rare Manifestation ◽  
Syndrome Diagnosis

Relapsing polychondritis (RP) is a rare autoimmune disease, characterized by recurrent inflammatory episodes affecting various carttilageus structures. Renal involvement in RP is unusual. Our case was a 42-year old woman presented with nephretic syndrome. Diagnosis of RP was made because of recurrent chondritis of both auricles, chondritis of costovertebral joints, non-erosive inflammatory arthritis of knees, and inflammation of ocular structures. Renal biopsy findings revealed diffuse crescentic and sclerotic glomerulonephritis, pauciimmune type. The diagnosis of RP may be missed when the destructive cartilage feature is not apparent or rare presentation of disease such as ocular or renal involvement preceded other manifestations. In this case we found that the irregular prednisolone use may prevent destructive cartilage damage but not renal failure of patient. We concluded that the association of crescentic glomerulonephritis in a patient with RP was assumed to be a rare manifestation of this disease.

scholarly journals Granulomatous nephritis: A rare presentation of juvenile-onset sarcoidosis

2021 ◽  
Author(s):  
Prashant Chotalia ◽  
Sapan Pandya ◽  
Puja Srivastava
Keyword(s):  
Renal Failure ◽  
Renal Involvement ◽  
Cervical Lymphadenopathy ◽  
Unknown Origin ◽  
Oral Prednisolone ◽  
Pulmonary Involvement ◽  
Juvenile Onset ◽  
Rare Presentation ◽  
Pyrexia Of Unknown Origin ◽  
Renal Functions

ABSTRACT Sarcoidosis is rare in children. Incidence and prevalence of sarcoidosis in India are not known. Renal involvement in childhood sarcoidosis is further rare with no clear data about prevalence. Here we report a case of a 13-year-old girl who presented with sarcoidosis with multi-system involvement including renal sarcoidosis. She initially presented with pyrexia of unknown origin and cervical lymphadenopathy – evaluation of which led to diagnosis of sarcoidosis. Later, after development of pulmonary involvement, she was treated with oral prednisolone and azathioprine. She again defaulted on medicines and later presented with renal failure and was diagnosed with a renal sarcoidosis. She was treated with oral prednisolone and mycophenolate mofetil with which she gradually improved with normal renal functions.

scholarly journals ANCA - Positive Churg Strauss Syndrome with Necrotizing Crescentic glomerulonephritis

JMS SKIMS ◽  
2009 ◽  
Vol 12 (1) ◽  
pp. 21-23
Author(s):  
A R Reshi ◽  
I A Wani ◽  
K A Banday ◽  
Iqbal Lone ◽  
M M Wani ◽  
...  
Keyword(s):  
Renal Failure ◽  
Renal Biopsy ◽  
Crescentic Glomerulonephritis ◽  
Renal Involvement ◽  
Eosinophilic Infiltration ◽  
Small Vessel Vasculitis ◽  
Churg Strauss Syndrome ◽  
Necrotizing Crescentic Glomerulonephritis ◽  
Strauss Syndrome ◽  
Churg Strauss

Churg Strauss Syndrome (CSS) is an ANCA associated small vessel vasculitis with eosinophilic infiltration. Renal involvement is frequent, although usually mild. We report a case of CSS with renal failure. Renal biopsy revealed necrotizing cresentic glomerulonephritis, eosonophilic arteritis and diffuses eosinophilic infiltration. Patient was successfully treated with combination of corticosteroids and cyclophosphamide. (JMed Sci 2009;12(1):21-23)

scholarly journals Microangiopathic Hemolytic Anemia and Fulminant Renal Failure: A Rare Manifestation of Pheochromocytoma

2019 ◽  
Vol 2019 ◽  
pp. 1-4
Author(s):  
Nishant Sharma ◽  
Divya Ravi ◽  
Mehvish Khan ◽  
Metlapalli Venkata Sravanthi ◽  
Mark M. Aloysius
Keyword(s):  
Renal Failure ◽  
Hemolytic Anemia ◽  
Young Male ◽  
Uncontrolled Hypertension ◽  
Microangiopathic Hemolytic Anemia ◽  
Rare Presentation ◽  
Paroxysmal Tachycardia ◽  
Rare Manifestation ◽  
Clinical Stabilization ◽  
Plasma Metanephrines

Pheochromocytoma is a rare adrenal tumor that is classically associated with the triad of paroxysmal tachycardia, diaphoresis, and headaches. However, it can have myriad manifestations. We present a case of a 31-year-old male who presented with abdominal pain, hypertensive emergency, and renal failure. Abdominal imaging demonstrated a left adrenal mass. Plasma metanephrines (153 pg/ml, n<57) and normetanephrines (1197 pg/ml, n<148) were noted to be elevated, leading to the diagnosis of pheochromocytoma. Intravenous antihypertensives were utilized to control his blood pressure. Hemodialysis was initiated given the degree of renal dysfunction. The patient subsequently developed hemolytic anemia, requiring the transfusion of multiple units of packed red cells. He developed acute respiratory failure leading to intubation, but was thereafter liberated from the ventilator following clinical stabilization. Uncontrolled hypertension precipitated by pheochromocytoma can cause microangiopathic hemolytic anemia and renal insufficiency. This case is notable not only for the occurrence of this rare presentation, but also for the severity of manifestations in a young male with no known significant comorbidities.

scholarly journals Typhoid Fever Presenting With Acute Renal Failure And Hepatitis Simultaneously - A Rare Presentation

2016 ◽  
Vol 22 (2) ◽  
pp. 80-82
Author(s):  
R. Rajput ◽  
D. Jain ◽  
A. Kumar ◽  
A. Mittal
Keyword(s):  
Developing Countries ◽  
Renal Failure ◽  
Acute Renal Failure ◽  
Typhoid Fever ◽  
Liver Enzymes ◽  
Renal Involvement ◽  
Rare Presentation ◽  
Hepatic Involvement ◽  
Pain Abdomen ◽  
Important Health

Abstract Typhoid fever is an important health problem worldwide but its incidence is more in developing countries. Hepatic involvement is common, but both hepatic and renal involvement is rare in typhoid fever. We report a case of typhoid fever presenting with hepatitis and acute renal failure. A 17 year old male presenting with fever and pain abdomen was found to have raised blood urea, creatinine, liver enzymes and bilirubin. Widal and typhidot (IgM,IgG) test were positive. His symptoms subsided and deranged parameters resolved with treatment of typhoid fever.

scholarly journals Bilateral Ureteral Stenosis with Hydronephrosis as First Manifestation of Granulomatosis with Polyangiitis (Wegener’s Granulomatosis): A Case Report and Review of the Literature

2020 ◽  
Vol 2020 ◽  
pp. 1-6
Author(s):  
Joelle Suillot ◽  
Jürg Bollmann ◽  
Samuel Rotman ◽  
Eric Descombes
Keyword(s):  
Wegener’S Granulomatosis ◽  
Crescentic Glomerulonephritis ◽  
Granulomatosis With Polyangiitis ◽  
Renal Involvement ◽  
Wegener's Granulomatosis ◽  
High Dose ◽  
Review Of The Literature ◽  
Ureteral Stenosis ◽  
Rare Manifestation ◽  
Unilateral Hydronephrosis

Ureteral stenosis is a rare manifestation of granulomatosis with polyangiitis (formerly known as Wegener’s granulomatosis). We report the case of a 76-year-old woman with progressive renal failure in which bilateral hydronephrosis due to ureteral stenosis was the first manifestation of the disease. Our patient also had renal involvement with pauci-immune crescentic glomerulonephritis associated with high titers of anti-proteinase 3 c-ANCAs, but no involvement of the upper or lower respiratory tract. The hydronephrosis and renal function rapidly improved under immunosuppressive therapy with high-dose corticosteroids and intravenous pulse cyclophosphamide. We reviewed the literature and found only ten other reported cases of granulomatosis with polyangiitis/Wegener’s granulomatosis and intrinsic ureteral stenosis: in two cases, the presenting clinical manifestation was unilateral hydronephrosis and in only two others was the hydronephrosis bilateral, but this complication developed during a relapse of the disease. This case emphasizes the importance of including ANCA-related vasculitis in the differential diagnosis of unusual cases of unilateral or bilateral ureteral stenosis.

Uremic Leontiasis Ossea: A Rare Presentation of Chronic Renal Failure Secondary to Hyperparathyroidism

2018 ◽  
Vol 2 (6) ◽  
Author(s):  
Ali Abdullah Almontasheri ◽  
Khalid Merheb Almutairi ◽  
Fayka Karem Ahmed ◽  
Ahmed Fathy Beh
Keyword(s):  
Renal Failure ◽  
Chronic Renal Failure ◽  
Rare Presentation

scholarly journals When systemic lupus erythematosus affects vision: a rare presentation of this condition

2020 ◽  
Vol 13 (1) ◽  
pp. e229382
Author(s):  
Tiago Gama Ramires ◽  
Luísa Vieira ◽  
Nuno Riso ◽  
Maria Francisca Moraes-Fontes
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Systemic Lupus ◽  
Blurred Vision ◽  
Rare Presentation ◽  
Complement Components ◽  
Oral Ulcers ◽  
Dsdna Antibody ◽  
Rare Manifestation ◽  
Retinal Vascular Tortuosity

A 23-year-old woman with fever, oral ulcers, arthalgias and weight loss of 2-week duration suddenly developed blurred vision, with reduced visual acuity, cotton wool exudates and retinal vascular tortuosity. Laboratory testing revealed anaemia, lymphopaenia, positive antinuclear antibody and high anti-dsDNA antibody titre with low complement components. There was no evidence of infection, clinching the diagnosis of lupus retinopathy. Steroid therapy alone was highly effective and was also accompanied by a normalisation of haemoglobin and lymphocyte counts, after which azathioprine was added. Hydroxychloroquine was introduced after resolution of retinal changes. Immunosuppressive therapy was progressively tapered over the course of 12 months and then discontinued, and the patient remains in remission 48 months after the initial presentation. Our patient exemplifies a very rare manifestation of systemic lupus erythematosus. We emphasise the importance of its early detection and complexity of treatment in order to reduce visual morbidity.

scholarly journals Lupus pernio (Besnier-Tenneson syndrome): A rare form of sarcoidosis

2018 ◽  
Vol 10 (2) ◽  
Author(s):  
Amal Hubail ◽  
Roza Belkharoeva ◽  
Natalya Tepluk ◽  
Tatyana Belerosova
Keyword(s):  
Psychological Effects ◽  
Management Approach ◽  
Cutaneous Sarcoidosis ◽  
Rare Form ◽  
Rare Presentation ◽  
Lupus Pernio ◽  
Rare Manifestation ◽  
Systemic Sarcoidosis ◽  
Delay In Treatment ◽  
Demographic Features

Lupus pernio (LP) is a chronic non-life threatining type of cutaneous sarcoidosis that can be related to chronic fibrotic sarcoidosis, hyperglobulinemia and hypercalcemia. The aim of this case report is to evaluate the clinical and demographic features of cutaneous sarcoidosis mainly presenting with a rare manifestation of LP. In this paper we report a case of systemic sarcoidosis presenting with LP and a review of the available literature. LP is a rare presentation with infiltrated erythematoviolaceous plaques affecting the nose. We address the main management approach, and possible association with an underlying systemic sarcoidosis. LP is a rare but chronic manifestation of systemic sarcoidosis that needs to be treated in order to prevent cosmetic defects and psychological effects. It is important to recognize such a condition early in order to avoid a delay in treatment and worsening of the condition, both physically and psychologically. Further research regarding the diagnostic approach and management is required to understand this condition thoroughly.

scholarly journals Anca-associated crescentic glomerulonephritis in a child with isolated renal involvement

2019 ◽  
Vol 41 (2) ◽  
pp. 293-295
Author(s):  
Mehtap Ezel Çelakıl ◽  
Burcu Bozkaya Yücel ◽  
Umay Kiraz Özod ◽  
Kenan Bek
Keyword(s):  
Crescentic Glomerulonephritis ◽  
Renal Involvement ◽  
Immunosuppressive Treatment ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Normal Serum ◽  
Laboratory Findings ◽  
Elevated Creatinine ◽  
Systemic Symptoms ◽  
Mild Clinical Presentation ◽  
Normal Serum Creatinine

ABSTRACT Pauci-immune glomerulonephritis (GN) is more common in elderly people compared to children and the etiology is not completely understood yet. Antineutrophil cytoplasmic antibody (ANCA) positivity occurs in 80% of the patients. We report a case of a 7-year-old girl who presented with malaise and mildly elevated creatinine diagnosed as ANCA-associated pauci-immune crescentic glomerulonephritis with crescents in 20 of 25 glomeruli (80%). Of these 20 crescents, 12 were cellular, 4 fibrocellular, and 4 globally sclerotic. She did not have purpura, arthritis, or systemic symptoms and she responded well to initial immunosuppressive treatment despite relatively severe histopathology. The patient was given three pulses of intravenous methylprednisolone (30 mg/kg on alternate days) initially and continued with cyclophosphamide (CYC; 2 mg/kg per day) orally for 3 months with prednisone (1 mg/kg per day). In one month, remission was achieved with normal serum creatinine and prednisone was gradually tapered. The case of this child with a relatively rare pediatric disease emphasizes the importance of early and aggressive immunosuppressive treatment in patients with renal-limited ANCA-associated pauci-immune crescentic GN even if with a mild clinical presentation. As in our patient, clinical and laboratory findings might not always exactly reflect the severity of renal histopathology and thus kidney biopsy is mandatory in such children to guide the clinical management and predict prognosis.

scholarly journals Renal involvement in human rabies: clinical manifestations and autopsy findings of nine cases from northeast of Brazil

2005 ◽  
Vol 47 (6) ◽  
pp. 315-320 ◽  
Author(s):  
Elizabeth De Francesco Daher ◽  
Geraldo Bezerra da Silva Júnior ◽  
Marúsia Thomaz Ferreira ◽  
Fernando Antonio de Sousa Barros ◽  
Tiago Magalhães Gurgel ◽  
...  
Keyword(s):  
Renal Failure ◽  
Acute Renal Failure ◽  
Renal Involvement ◽  
Clinical Manifestations ◽  
Signs And Symptoms ◽  
Hemodynamic Instability ◽  
Human Rabies ◽  
Tubular Necrosis ◽  
Post Mortem Findings ◽  
Histopathologic Findings

A retrospective study was conducted in nine patients with rabies admitted to a hospital of Fortaleza, Brazil. Autopsy was performed in all cases. The ages ranged from three to 81 years and six were males. They all were bitten by dogs. The time between the accident and the hospital admission ranged from 20 to 120 days (mean 45 ± 34 days). The time until death ranged from one to nine days (mean 3.3 ± 5.5 days). The signs and symptoms presented were fever, hydrophobia, aerophobia, agitation, disorientation, dyspnea, sialorrhea, vomiting, oliguria, sore throat, pain and hypoesthesia in the site of the bite, headache, syncope, cough, hematemesis, mydriasis, hematuria, constipation, cervical pain and priapism. In three out of six patients, there was evidence of acute renal failure, defined as serum creatinine > 1.4 mg/dL. The post-mortem findings in the kidneys were mild to moderate glomerular congestion and mild to intense peritubular capillary congestion. Acute tubular necrosis was seen in only two cases. This study shows some evidence of renal involvement in rabies. Histopathologic findings are nonspecific, so hemodynamic instability, caused by autonomic dysfunction, hydrophobia and dehydration must be responsible for acute renal failure in rabies.

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