- Standardized classification and reporting of glomerulonephritis. [Journal Article]
- NDNephrol Dial Transplant 2018 Aug 13
- A kidney biopsy is done to determine the etiology of the glomerulonephritis (GN) and the severity of the lesion, to identify whether other lesions, related to or not related to the GN, are present on...
A kidney biopsy is done to determine the etiology of the glomerulonephritis (GN) and the severity of the lesion, to identify whether other lesions, related to or not related to the GN, are present on the kidney biopsy and finally to ascertain the extent of chronicity of the GN. The etiology of GN is based on the classification of GN into five groups: immune complex-mediated GN, antineutrophil cytoplasmic antibody (ANCA)-associated GN, anti-glomerular basement membrane (GBM) GN, monoclonal immunoglobulin-mediated GN and C3 glomerulopathy. Immune complex GN includes multiple specific diseases such as lupus nephritis, IgA nephropathy, infection-related GN and fibrillary GN. ANCA GN, anti-GBM GN and C3 glomerulopathy are specific diseases in themselves, while monoclonal Ig GN includes proliferative GN with monoclonal Ig deposits and monoclonal Ig deposition disease. Thus identification of the class of GN and within it the specific disease determines the etiology of GN. Ancillary studies may be required to confirm the etiology of GN. The severity of the GN is revealed by the pattern of injury, such as crescentic, necrotizing, diffuse proliferative, exudative, membranoproliferative, mesangial proliferative or a sclerosing GN. Secondary diagnosis either related or unrelated to the GN, such as diabetic glomerulosclerosis, acute tubular necrosis or thrombotic microangiopathy, may also be present. The secondary diagnosis may sometimes be the reason for the kidney biopsy. The chronicity of GN is determined by evaluating the extent of glomerulosclerosis, tubular atrophy and interstitial fibrosis and vascular sclerosis present on the biopsy. This review summarizes the approach to standardizing a kidney biopsy report that includes these components in a logical and sequential manner.
- Case 24-2018: A 71-Year-Old Man with Acute Renal Failure and Hematuria. [Case Reports]
- NEJMN Engl J Med 2018 Aug 09; 379(6):568-578
- Double-positive with positive anti-glomerular basement membrane antibody and ANCA-positive disease in a patient with dermatomyositis. [Journal Article]
- BCBMJ Case Rep 2018 Jul 24; 2018
- Approximately one in four patients (23.3%) with inflammatory myositis including dermatomyositis can require evaluation for acute kidney injury. The main cause of kidney injury is acute tubular necros...
Approximately one in four patients (23.3%) with inflammatory myositis including dermatomyositis can require evaluation for acute kidney injury. The main cause of kidney injury is acute tubular necrosis from medications or myoglobinuria, though clinicians should be aware of a wide variety of possible aetiologies. We present the case of a 44-year-old woman with stable anti-Jo1 dermatomyositis, who presented with acute kidney injury. During her hospital course, she was diagnosed with double-positive disease characterised by circulating anti-glomerular basement membrane antibody and myeloperoxidase antineutrophil cytoplasmic antibody and renal biopsy revealing crescentic glomerulonephritis with linear staining of capillary wall for IgG.
- Goodpasture's disease in a patient with advanced lung cancer treated with nivolumab: An autopsy case report. [Journal Article]
- LCLung Cancer 2018; 122:22-24
- Nivolumab, an anti-programmed death-1 immune checkpoint inhibitor (ICI), is now widely used to treat numerous cancers. Although most adverse effects related to ICIs are controllable, fulminant immune...
Nivolumab, an anti-programmed death-1 immune checkpoint inhibitor (ICI), is now widely used to treat numerous cancers. Although most adverse effects related to ICIs are controllable, fulminant immune-related adverse events can occur. A 74-year-old patient with non-small-cell lung cancer was treated with nivolumab as a second-line treatment. After 8 cycles, acute kidney injury with macroscopic hematuria appeared, followed by diffuse ground-glass opacities with hemoptysis. Since the clinical course suggested Goodpasture's disease, methylprednisolone pulse therapy and plasma exchange were started. Later, it was confirmed that the serum anti-glomerular basement membrane antibody was positive. However, the patient died 35 days after admission due to respiratory failure, and an autopsy showed crescentic glomerulonephritis and massive alveolar hemorrhage which were compatible with Goodpasture's disease. Our case provides a possible link between nivolumab and lethal Goodpasture's disease.
- Stability of important antibodies for kidney disease: pre-analytic methodological considerations. [Journal Article]
- PPeerJ 2018; 6:e5178
- The importance of circulating antibodies as biomarkers of kidney disease has recently been recognized. However, no study has systematically described the methodology of sample preparation and storage...
The importance of circulating antibodies as biomarkers of kidney disease has recently been recognized. However, no study has systematically described the methodology of sample preparation and storage regarding antibodies as biomarkers of kidney disease. It remains unknown whether repetitive freeze-thaw cycles, physical disturbances, storage at different temperatures or for different periods of time, or haemolytic or turbid serum samples affect antibody measurements. The aim of this study was to investigate the stabilities of antibodies associated with kidney disease in serum samples under various relevant clinical and research conditions.
- Prevalence of overlap of antineutrophil cytoplasmic antibody associated vasculitis with systemic autoimmune diseases: an unrecognized example of poliautoimmunity. [Journal Article]
- CRClin Rheumatol 2018 Jul 14
- We aimed to estimate the frequency of overlap of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) with systemic autoimmune diseases. Retrospective single-center study to identif...
We aimed to estimate the frequency of overlap of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) with systemic autoimmune diseases. Retrospective single-center study to identify patients with AAV diagnosis and concomitant autoimmune systemic diseases, simultaneously, before or after the diagnosis of AAV. Sociodemographic characteristics, such as comorbidities; follow-up time; type of AAV; disease duration; relapses; treatment and response; clinical, serological, and histological characteristics; disease activity and damage; prognosis; dialysis requirements, and death were assessed. Twenty-eight of two hundred and forty-seven patients (11.3%) with AAV had a concomitant diagnosis of autoimmune disease. The predominant AAV type was renal-limited vasculitis (39%), followed by granulomatosis with polyangiitis (29%), mycroscopic polyangiitis (25%), and eosinophilic granulomatosis with polyangiitis (7%). Mean age at AAV diagnosis was 50 ± 17 years and 24/28 were ANCA positive. The main clinical manifestations were renal (79%), otorhinolaryngologic (43%), and pulmonary and peripheral neuropathy (32%). Sixteen patients (57%) experienced partial or total remission at a median follow-up of 34 months, and four patients (14%) died. The most frequent autoimmune disease overlapped was rheumatoid arthritis (39%), followed by Sjögren's syndrome and systemic sclerosis (14%), mixed connective tissue disease (11%), systemic lupus erythematosus and juvenile idiopathic arthritis (7%), and ankylosing spondylitis and IgG4-related disease (4%). In nine patients (32%), both diagnoses were simultaneous; in the rest, median time elapsed between the autoimmune disease and AAV diagnosis was 173 months. The prevalence of overlap AAV with other autoimmune diseases was low. The most common AAV phenotype was renal-limited vasculitis, and the most frequent overlap disease was rheumatoid arthritis.
- The Respiratory System in Autoimmune Vascular Diseases. [Journal Article]
- RRespiration 2018; 96(1):12-28
- The respiratory system may be involved in all types of systemic vasculitis with varying significance and frequency. ANCA-associated vasculitis, including granulomatosis with polyangiitis, eosinophili...
The respiratory system may be involved in all types of systemic vasculitis with varying significance and frequency. ANCA-associated vasculitis, including granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, and microscopic polyangiitis, affects the respiratory tract more commonly than other vasculitis types. Granulomatosis with polyangiitis is always associated with upper or lower respiratory tract involvement. Pulmonary and ENT involvements are the hallmark feature of the disease and are present in 90 and 80% of cases, respectively, with frequent skin or gastrointestinal involvement. In about 10% of cases, the lung is the only organ affected. Eosinophilic granulomatosis with polyangiitis is always associated with hypereosinophilia and asthma which usually precedes the systemic manifestations by several years; however, onset of asthma and of the vasculitis may be concomitant. Parenchymal infiltrates may be migratory and rapidly resolve upon corticosteroid treatment. Diffuse alveolar hemorrhage and renal failure are typical features of microscopic polyangiitis. The former is the leading manifestation of anti-glomerular basement membrane disease and is usually part of a pulmonary-renal syndrome. Takayasu arteritis has a distinct clinical presentation due to pulmonary arteritis and may present with massive hemoptysis, chest pain, and rarely symptoms of pulmonary hypertension. Behçet disease is the most common cause of pulmonary artery aneurysm and can also cause in situ thrombosis of the pulmonary arteries. Corticosteroids and immunosuppressive agents are the mainstay of treatment. In conclusion, systemic vasculitis is a frequent cause of respiratory system involvement with diverse manifestations of distinct severity and outcome.
- Adult onset immunoglobulin A vasculitis (Henoch-Schonlein purpura) with alveolar hemorrhage. [Journal Article]
- IIDCases 2018; 12:47-48
- S. pyogenes is the cause of many important human diseases, ranging from mild superficial skin infections to life-threatening systemic diseases. The post streptococcal syndromes are immune mediated ph...
S. pyogenes is the cause of many important human diseases, ranging from mild superficial skin infections to life-threatening systemic diseases. The post streptococcal syndromes are immune mediated phenomena including Immunoglobulin A Vasculitis (Henoch-Schönlein purpura). HSP is more common in children and usually self limited but it can cause skin, joint, renal, gastrointestinal and rarely respiratory involvement. We present a case with Streptococcus pyogenes pneumonia that presented with respiratory failure, pulmonary hemorrhage, extensive rash and renal failure.
- Primary membranous nephropathy presenting with crescentic glomerulonephritis 25 years after initial presentation: A case report . [Journal Article]
- CNClin Nephrol 2018 Jun 22
- Membranous nephropathy (MN) is a common cause of nephrotic syndrome. Rarely, it can present with rapidly-progressive renal failure and hematuria. While this may be due to lupus nephritis, superimpose...
Membranous nephropathy (MN) is a common cause of nephrotic syndrome. Rarely, it can present with rapidly-progressive renal failure and hematuria. While this may be due to lupus nephritis, superimposed anti-glomerular basement membrane (GBM) disease, or antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, there have been rare reports of anti-GBM - and ANCA-negative crescentic glomerulonephritis presenting in primary membranous nephropathy (pMN). We present the case of a patient with long-standing pMN who developed acute deterioration of renal function and was found to have a flare of MN along with crescentic glomerulonephritis (GN) despite a negative serum ANCA, anti-GBM, and antinuclear antibody (ANA) work-up. He was started on dialysis and immunosuppressive therapy, and eventually recovered enough renal function to become dialysis-independent. A brief review of available literature suggests that crescentic GN presenting with pMN is a rare but established entity. Much more rarely has it been reported to occur in patients with previously-diagnosed pMN. In these contexts, crescentic GN may be occurring as the most severe manifestation of pMN rather than as a separate entity. Immunosuppressive therapy is often given, however, prognosis is guarded as half of patients will have worsening renal function and a quarter will develop end-stage renal disease. .
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- Impairment of gamma-glutamyl transferase 1 activity in the metabolic pathogenesis of chromophobe renal cell carcinoma. [Journal Article]
- PNProc Natl Acad Sci U S A 2018 Jul 03; 115(27):E6274-E6282
- Chromophobe renal cell carcinoma (ChRCC) accounts for 5% of all sporadic renal cancers and can also occur in genetic syndromes including Birt-Hogg-Dube (BHD) and tuberous sclerosis complex (TSC). ChR...
Chromophobe renal cell carcinoma (ChRCC) accounts for 5% of all sporadic renal cancers and can also occur in genetic syndromes including Birt-Hogg-Dube (BHD) and tuberous sclerosis complex (TSC). ChRCC has a distinct accumulation of abnormal mitochondria, accompanied by characteristic chromosomal imbalances and relatively few "driver" mutations. Metabolomic profiling of ChRCC and oncocytomas (benign renal tumors that share pathological features with ChRCC) revealed both similarities and differences between these tumor types, with principal component analysis (PCA) showing a distinct separation. ChRCC have a striking decrease in intermediates of the glutathione salvage pathway (also known as the gamma-glutamyl cycle) compared with adjacent normal kidney, as well as significant changes in glycolytic and pentose phosphate pathway intermediates. We also found that gamma glutamyl transferase 1 (GGT1), the key enzyme of the gamma-glutamyl cycle, is expressed at ∼100-fold lower levels in ChRCC compared with normal kidney, while no change in GGT1 expression was found in clear cell RCC (ccRCC). Significant differences in specific metabolite abundance were found in ChRCC vs. ccRCC, including the oxidative stress marker ophthalmate. Down-regulation of GGT1 enhanced the sensitivity to oxidative stress and treatment with buthionine sulfoximine (BSO), which was associated with changes in glutathione-pathway metabolites. These data indicate that impairment of the glutathione salvage pathway, associated with enhanced oxidative stress, may have key therapeutic implications for this rare tumor type for which there are currently no specific targeted therapies.