Case Of IgA Nephropathy Research Articles

A Novel Case of IgA Nephropathy and Tubulointerstitial Nephritis in Patients with Crohn Disease Treated with Adalimumab: What Is the Culprit?

A Novel Case of IgA Nephropathy and Tubulointerstitial Nephritis in Patients with Crohn Disease Treated with Adalimumab: What Is the Culprit?

A Unique Case of IgA Nephropathy Associated with Both Immune Thrombocytopenia and Erythrocytosis

A Unique Case of IgA Nephropathy Associated with Both Immune Thrombocytopenia and Erythrocytosis

Outcome of Glomerular Disease Manifesting After Vaccination Against Severe Acute Respiratory Syndrome Coronavirus 2.

Introduction Vaccination against severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) can upregulate the immune system and may contribute to glomerular disease (GD). Here, we describe a spectrum of GD that manifested following vaccination against SARS-CoV-2 (COVID-19 vaccinations). Material and methods This was a descriptive study of 10 cases enrolled between January 2021 and January 2023. Patients with biopsy-proven GD that manifested following COVID-19 vaccinations were included. Results We found 10 cases of biopsy-proven GD following the COVID-19 vaccination. This included five cases of minimal change disease (MCD), three cases of focal segmental glomerulosclerosis (FSGS), one case of C3 glomerulonephritis (C3GN), and one case of IgA nephropathy (IgAN). The pre-existing disease was found in the last two patients (IgAN and C3GN) who got unmasked following vaccination. We did not observe any relation between vaccine type (Covisheld; six cases vs. Covaxin; four cases) and GD. In most cases (8/10 cases, 80.0%), GD developed after a repeat dose (second or booster dose). The onset time following vaccination was typically less than a week, and even less following a repeat dose. Conclusion Post-vaccination GD can be either de novo or a flare-up of a pre-existing one. The onset time following vaccination was typically less than a week for both Covishield and Covaxin.

A case diagnosed with IgA nephropathy during a complete remission of minimal change nephrotic syndrome treated with rituximab.

We herein report a case of IgA nephropathy in a 20-year-old male who maintained a complete remission of minimal change nephrotic syndrome (MCNS) through the administration of rituximab (RTX). He was diagnosed with nephrotic syndrome at 4years of age. After he relapsed frequently, he was diagnosed with MCNS at 8years of age based on the findings of a kidney biopsy. At 13years of age, RTX therapy was initiated to maintain a complete remission after steroid treatment. MCNS recurred twice, including the time in which the interval between the RTX administrations was long. Whenever he relapsed, remission induction was achieved using steroids, and remission maintenance was achieved using RTX. Five months after the 7th RTX administration, the serum IgA level started to increase. After the 9th RTX administration, he demonstrated microhematuria despite the urinary protein level indicating complete remission. At the 10th administration, the urinary protein and the red-blood cell casts were also observed. A renal biopsy was performed 84months after the initial administration of RTX, and the patient was diagnosed with complications of IgA nephropathy. RTX is not considered to be a useful treatment for IgA nephropathy. The reasons for this are due to the fact that IgA1 does not decrease even following the administration of RTX, because B cells residing in the mucosa may not be deleted by RTX, and IgA production may also continue due to the presence of CD20- long-lived plasma cells. Even when administering RTX, if there are findings of glomerulonephritis on urine testing, the possibility of IgA nephropathy must be considered.

IgA nephropathy in a child with X-linked agammaglobulinemia: a case report

BackgroundX-linked agammaglobulinemia (XLA) is a primary immunodeficiency disease caused by mutations in the Bruton tyrosine kinase (BTK) gene. Individuals diagnosed with XLA are at an increased risk of developing autoimmune diseases. However, renal involvement are rare in cases of XLA.Case presentationIn this report, we discussed a specific case involving a 6-year-old boy with XLA who experienced recurrent upper respiratory tract infections since the age of one. He presented with symptoms of hematuria and proteinuria, and renal pathology confirmed the presence of immunoglobulin (Ig) A nephropathy. Treatment comprised glucocorticoids, mycophenolate mofetil, and intermittent intravenous immunoglobulin replacement therapy. Consequently, there was a remission of proteinuria and a partial improvement in hematuria.ConclusionsIn this study, we describe the first case of IgA nephropathy associated with XLA. This is an interesting phenotype found in XLA, and it provides valuable insights into the process of autoimmunity and the regulation of immune function in individuals with XLA. Based on our findings, we recommend the evaluation of immunoglobulin levels in patients diagnosed with IgA nephropathy.

Recurrence of Glomerulonephritis After Kidney Transplantation - Experience of One Center from 2020 to 2023

Glomerulonephritis (GN) after kidney transplantation is a common problem. Many of them are recurrences of the primary disease in the transplanted kidney. The course and prognosis of individual types of glomerulonephritis (GN) are very different and their appearance may worsen the graft survival. World statistics show significant discrepancies regarding the incidence of GN recurrence depending on the adopted protocol (protocolar biopsy or due to symptoms). We analyzed the transplanted kidney biopsy results that are performed only in symptomatic patients. A group of 125 patients transplanted and treated in one medical center were observed. In this group, in 32 patients, the primary kidney disease was GN, confirmed by kidney biopsy before transplantation. Twenty three kidney biopsies were performed; in 8, cases the primary disease was GN. The indication for biopsy were hematuria and/or proteinuria and/or graft failure. We diagnosed 5 cases of GN, including 4 cases of GN recurrence (12.5% in whole GN group, 50% in symptomatic GN group). In the relapse group, there was 1 case of IgA nephropathy (the earliest recurrence 1 month after transplantation), 1 case of focal segmental glomerulosclerosis, 1 case of membranous nephropathy, and 1 case of lupus nephritis (the latest recurrence 1 year and 4 months after transplantation). Our observation showed a high percentage of GN recurrences in symptomatic patients. This indicates the need to specify data regarding the diagnosis of recurrence depending on the adopted research method (protocolar or due to symptoms biopsy) to know which patients should be treated.

Exploring kidney biopsy findings in congenital heart diseases: Insights beyond cyanotic nephropathy.

The association between congenital heart disease and chronic kidney disease is well known. Various mechanisms of kidney damage associated with congenital heart disease have been established. The etiology of kidneydisease has commonly been considered to be secondary to focal segmental glomerulosclerosis (FSGS), however, this has only been demonstrated in case reports and not in observational or clinical trials. To identify baseline and clinical characteristics, as well as the findings in kidney biopsies of patients with congenital heart disease in our hospital. This is a retrospective observational study conducted at the Nephrology Department of the National Institute of Cardiology "Ignacio Chávez". All patients over 16 years old who underwent percutaneous kidney biopsy from January 2000 to January 2023 with congenital heart disease were included in the study. Ten patients with congenital heart disease and kidney biopsy were found. The average age was 29.00 years ± 15.87 years with pre-biopsy proteinuria of 6193 mg/24 h ± 6165 mg/24 h. The most common congenital heart disease was Fallot's tetralogy with 2 cases (20%) and ventricular septal defect with 2 (20%) cases. Among the 10 cases, one case of IgA nephropathy and one case of membranoproliferative glomerulonephritis associated with immune complexes were found, receiving specific treatment after histopathological diagnosis, delaying the initiation of kidney replacement therapy. Among remaining 8 cases (80%), one case of FSGS with perihilar variety was found, while the other 7 cases were non-specific FSGS. Determining the cause of chronic kidney disease can help in delaying the need for kidney replacement therapy. In 2 out of 10 patients in our study, interventions were performed, and initiation of kidney replacement therapy was delayed. Prospective studies are needed to determine the usefulness of kidney biopsy in patients with congenital heart disease.

Known case of ESRD due to IgA nephropathy with myelodysplastic syndrome having perinephric hematoma and disseminated multi-drug resistant Klebsiella pneumonia infection in urinary tract, gastrointestinal tract and testes following living donor kidney transplantation: The strength of multidisciplinary team management

A 34 years old man, had increasing pain over grafted kidney with low urine output on post-operative ‘Day 7’ following living donor kidney transplantation. He was a known case of IgA nephropathy with end stage renal disease and Myelodysplastic Syndrome (MDS). CT abdomen revealed perinephric hematoma; cast effect on graft kidney; and the density over grafted kidney was decreased suggestive of rejection. He also had disseminated multi-drug resistant Klebsiella pneumonia infections in urinary tract, gastrointestinal tract and orchitis. Multi-disciplinary team’s management which includes nephrologists, general physicians, hematologists, uro-surgeons, vascular surgeons, radiologists, microbiologists and anesthetists saves patient’s life as well as transplant kidney.

#6795 DISSEMINATED STRONGYLOIDES INFECTION IN A PATIENT WITH IGA NEPHROPATHY ON IMMUNOSUPPRESSIVE MEDICATIONS

Abstract Background and Aims Immunosuppressive medications such as high dose steroids and antimetabolites are frequently used in glomerulonephritis. However, use of these medications is usually associated with various adverse effects. Among the drawbacks of immunosuppressive therapy, the risk of reactivation of latent infections, including parasitic infestations, is to be considered. Method Case report. Results Our case is a 28 year old male patient who was diagnosed as a case of IgA nephropathy 7 months back with M1E1S1T1C0 lesions with a serum creatinine of 1.7 mg/dL and eGFR of 56 ml/min/1.73 m2. He was started on prednisolone (40mg daily) and mycophenolate mofetil (1000mg twice daily) for 1 month. He presented with us with diarrhea. He was treated with IV fluids and mycophenolate mofetil was stopped. After two weeks, he was readmitted with a 4-day history of fever, vomiting, and epigastric pain. On examination, he showed mild epigastric tenderness, normal vitals. The laboratory investigations on admission showed hemoglobin-15 g/dL, total leucocyte count-8600, eosinophilia with absolute eosinophil count 1900/ mm3, urea-92 mg/dL, creatinine-2 mg/dL. ECG showed sinus tachycardia, and HRCT chest revealed bilateral ground glass opacities and a calcified nodule in the left upper lobe. Liver function test was normal with normal amylase and calcium level. Blood culture was sterile. Ultrasound abdomen showed a bilateral raised Eco pattern in kidneys rest were normal. Upper GI endoscopy revealed diffuse edema, erythema, and superficial ulceration in duodenum and biopsy was taken. The duodenal biopsy showed features of strongyloidiasis stercoralis (Figure 1). Bronchoesophageal lavage was done which revealed strongyloidiasis stercoralis larvae. Meanwhile, the patient developed subacute small bowel obstruction with abdominal x ray showing multiple air fluid levels and CT scan showed features of small bowel obstruction with transition at mid-ileal level secondary to short segment thickening. Small bowel obstruction was relived with conservative mangement with ryles tube decompression and enemas. He was diagnosed with disseminated strongyloidiasis and treated with ivermectin for 14 days with full recovery Conclusion Our case signifies need of awareness among clinicians of the risk of disseminated strongyloidiasis as a complication of the treatment with immunosuppressive medications even in non-endemic areas.

Alport syndrome misdiagnosed with IgA nephropathy from familial history: a case report and brief review

BackgroundAlport syndrome is a rare inherited disease resulting from a primary disorder of the glomerular basement membrane. This disease results from mutations in genes encoding alpha chains of type IV collagen. In the differential diagnosis of this disease, IgA nephropathy is the most common primary glomerular disease with gross or microscopic hematuria.Case presentationA 50-year-old woman was presented with microscopic hematuria and proteinuria of under one gram. Due to the diagnosis of IgA nephropathy in family members, she was treated and followed up for 4 years as a possible case of IgA nephropathy. Eye examination and audiometry were normal. She underwent renal biopsy with an exacerbation of proteinuria. There was no finding in favor of IgA nephropathy in the histological examination, but the findings of electron microscopy and family history favored Alport syndrome.ConclusionsThis case demonstrates the importance of accurate history and electron microscopy in the complete histological evaluation and diagnosis of glomerular disease. Although in most cases the two can be differentiated based on clinical manifestations, laboratory findings, and histopathological examination, sometimes the association of these two diseases in the families involved or the lack of accurate history and complete histological examinations can complicate the diagnosis.

A case report of aneurysmal subarachnoid hemorrhage in a Young with IgA: A mere co-existence?

Introduction and importanceAneurysmal Subarachnoid hemorrhage (SAH) can be associated with various other conditions like Polycystic kidney disease, Ehler Danlos syndrome, Co-arctation of the aorta, etc.Case presentationHere we have reported a 21-year young lady with IgA nephropathy and aneurysmal SAH managed successfully.Clinical findings and investigationsThe patient was a known case of IgA nephropathy diagnosed 18 months back from a kidney biopsy. She came to the emergency with a headache and vomiting for 2 weeks. Her initial headache was the worst she has had experienced and then the headache was continuous and not relieved by medications. Then she underwent a noncontrast CT scan of the head which showed Subarachnoid hemorrhage (SAH).Interventions and outcomesLeft pterional craniotomy and microsurgical clipping of left ICA aneurysm performed. The patient was discharged without deficits.Relevance and impactAneurysmal Subarachnoid hemorrhage(SAH) can be due to histopathological changes to the vessels following IgA nephropathy. These correlations could be studied further or else it could be only a mere coincidence.

De Novo versus Relapse of Glomerulonephritis Post COVID-19 Vaccination: Case Series in a Single Center in Saudi Arabia

The development of de novo or relapse of an already existing glomerular disease has been reported in association with some COVID-19 vaccinations, specifically the mRNA vaccines. Although reported cases are sparse and the number of reported cases are considered low in comparison to the total number of administered vaccine doses worldwide, the healthcare providers as well as the patients should be aware about such a serious complication in order to provide prompt and proper management. Here we report 3 cases of glomerular diseases in relation to COVID-19 vaccination. One case of IgA nephropathy, a case of IgA vacuities and nephropathy and a case of a relapse of a minimal change disease.

Association of ulcerative colitis and IgA nephropathy: A case report

The extradigestive manifestations of chronic inflammatory bowel disease most often affect the articulations, skin, eyes, liver and bile ducts. Renal involvement is rare, and manifests as kidney stones, glomerulonephritis, tubulointerstitial nephritis, and secondary amyloidosis. In this context of chronic inflammatory bowel disease, in particular ulcerative colitis, renal involvement is very often secondary to nephrotoxicity of the basic treatment of digestive pathology, and very rarely an authentic extradigestive manifestation of intestinal disease. We report a case of IgA nephropathy as an extra-digestive manifestation of ulcerative colitis. The objective of this study is not to neglect the IgA nephtopathy as an extradigestive manifestation of IBD which, even though rare, remains a condition to be looked for by clinicians during the follow-up of IBD.

Tonsillectomy in IgA Nephropathy - Clinical Case

Abstract This article presents the clinical case of IgA nephropathy of a patient hospitalized in order to investigate a nephritic syndrome, apparently with acute onset, discovered in the following circumstances: recurrent macroscopic hematuria, decreased urinary volume, the first episode occurring about a month ago in the context of an acute infection of upper airways, remitted under antibiotic therapy, with reappearance every 2 weeks, also accompanied by odynophagia and decreased urinary volume.

Association of Ulcerative Colitis and IgA Nephropathy: A Case Report

The extra-digestive manifestations of chronic inflammatory bowel disease most often affect the joints, skin, eyes, liver and bile ducts. Renal involvement is rare, and manifests as kidney stones, glomerulonephritis, tubulointerstitial nephritis, and AA-type secondary amyloidosis. In this context of chronic inflammatory bowel disease, in particular ulcerative colitis, renal involvement is very often secondary to nephrotoxicity of the basic treatment of digestive pathology, and very rarely an authentic extra-digestive manifestation of intestinal disease. We report a case of IgA nephropathy as an extra-digestive manifestation of ulcerative colitis.

ANCA- positive IgA nephropathy presented as alveolar hemorrhage in a COVID-19 patient

Coronavirus disease 2019 (COVID-19) is a global pandemic caused by Severe Acute Respiratory Syndrome Coronavirus-2 (SARS-CoV-2). The SARS-CoV-2 virus primarily targets the respiratory system, but extrapulmonary involvements can be frequently seen. We presented a COVID-19 case with anti-neutrophil cytoplasmic antibody-positive IgA nephropathy, alveolar hemorrhage, and rapidly progressive kidney disease. The patient received pulse corticosteroids, plasma exchange, and intravenous immunoglobulin as treatment. Azathioprine was added as an immunosuppressive therapy. To the best of our knowledge, this is the first reported case of IgA nephropathy coexisting with COVID-19 infection.

An Unusual Case of IgA Nephropathy Associated with Parvovirus B19

An Unusual Case of IgA Nephropathy Associated with Parvovirus B19

IgA nephropathy in a patient receiving infliximab for generalized pustular psoriasis

BackgroundIgA nephropathy is the most common glomerulonephritis. Secondary IgA nephropathy complicated with systemic diseases, including psoriasis, is also often reported. Generalized pustular psoriasis is a form of psoriasis characterized by sterile pustules on reddened skin and fever. Infliximab, one of the first-line therapies for severe psoriasis, has also been reported to cause systemic vasculitis and IgA nephropathy. We herein report a case of IgA nephropathy activated during infliximab treatment for generalized pustular psoriasis.Case presentationA 28-year-old woman presented with episodic gross hematuria, increasing proteinuria, and renal dysfunction. She had been receiving anti-TNFα therapy with infliximab because of generalized pustular psoriasis for 3 years, but her skin symptoms worsened following withdrawal during pregnancy. After delivery, her skin symptoms improved with the resumption of infliximab, but clinical signs suggested glomerulonephritis, and renal biopsy showed active IgA nephropathy. Infliximab was discontinued, and the combination of corticosteroids, tonsillectomy, and secukinumab, an IL-17A inhibitor, improved both the skin symptoms and the glomerulonephritis.ConclusionsIn our case, the activity of IgA nephropathy was exacerbated by anti-TNFα therapy but was improved by the combination of corticosteroids, tonsillectomy, and an IL-17A inhibitor against the original disease. Autoimmune diseases may underlie the development of secondary IgA nephropathy associated with anti-TNFα therapy, and so further studies are needed to better understand the association between molecular-targeted drugs and IgA nephropathy.

A Pediatric Case of IgA Nephropathy Associated with Familial Mediterranean Fever

IgA nephropathy (IgAN) is one of the most common primary glomerulonephritis in children and adolescents. Pharyngitis infections are known to be a trigger of IgA attacks. It has been suggested that IgAN is an immune complex disease with circulating immune complexes galactose-deficient IgA1 (Gd-IgA1), IgG and IgA anti-Gd-IgA1 antibodies, and soluble CD89. Amyloidosis is the most common renal complication of Familial Mediterranean Fever (FMF), the most common form of auto-inflammatory syndromes characterized by flares of fever and polyserositis. Besides amyloidosis, other renal lesions have been exceptionally reported in patients with FMF and other hereditary periodic fevers. We report a child who presented with haematuria during febrile attacks in the context of FMF, and in whom kidney biopsy revealed deposits of predominant mesangial IgA with no evidence of amyloidosis and presence of immune complexes in the serum (Gd-IgA and sCD89-IgA).

Diagnostic role of renal biopsy in PLA2R1-antibody-positive patients with nephrotic syndrome

Renal biopsy is the gold standard for diagnosis of membranous nephropathy. Circulating PLA2R1 antibody found in 75% of patients with membranous nephropathy is very specific for the diagnosis of this disease. Therefore, the question arises whether PLA2R1-antibody-positive patients still need a diagnostic renal biopsy. In this study we investigated whether additional relevant information is obtained by performing renal biopsy in nephrotic patients, who are PLA2R1-antibody positive. A detailed analysis of renal biopsies, including immunohistochemistry and electron microscopy, was performed in 263 patients with biopsy-proven membranous nephropathy, of whom 194 patients were PLA2R1-antibody positive, to detect diagnostic features additional to membranous nephropathy. Twelve (6%) of the 194 PLA2R1-antibody-positive patients had a second relevant diagnosis in addition to membranous nephropathy: five (3%) patients had interstitial nephritis, in five (3%) other patients a diabetic nephropathy was diagnosed and two (1%) patients had IgA nephropathy. Patients with a second diagnosis in addition to membranous nephropathy had a significantly higher serum creatinine (p < 0.01) and lower eGFR (p = 0.04) compared to patients in whom only the diagnosis of membranous nephropathy was made. In 7 (10%) of 69 PLA2R1-antibody-negative patients, renal biopsies showed an additional diagnosis to membranous nephropathy: one (1%) case of IgA nephropathy, cholesterol emboli, IgG4-related disease, necrotising glomerulonephritis, thrombotic microangiopathy, interstitial nephritis and diabetic nephropathy each. The advantage of detecting an additional diagnosis to membranous nephropathy in 6% of PLA2R1-antibody-positive patients by renal biopsy has to be balanced to the potential risks and costs of the biopsy procedure. Renal biopsy is particularly relevant in patients presenting with impaired renal function and abnormalities in urinalysis going beyond proteinuria. Immunohistochemical staining for PLA2R1 was the only histomorphologic analysis allowing a reliable differentiation of PLA2R1-antibody-positive from PLA2R1-antibody-negative membranous nephropathy.