obsolete acute glomerulonephritis with lesion of rapidly progressive glomerulonephritis

Obsolete Acute Glomerulonephritis and Rapidly Progressive Glomerulonephritis

Acute glomerulonephritis, also known as post-streptococcal glomerulonephritis (PSGN), is a condition that was once considered an acute presentation of rapidly progressive glomerulonephritis. However, it has been reclassified as a distinct entity due to its different clinical and pathological features.

Characteristics

Obsolete acute glomerulonephritis with lesion of rapidly progressive glomerulonephritis presents with:

• Rapid decline in kidney function: A rapid loss of renal function over a short period, often within days or weeks.
• Hematuria and proteinuria: Presence of blood and protein in the urine.
• Crescent formation: The presence of crescents in > 50% of glomeruli, which is a hallmark of rapidly progressive glomerulonephritis.

Differences from Rapidly Progressive Glomerulonephritis

While obsolete acute glomerulonephritis shares some similarities with rapidly progressive glomerulonephritis, it has distinct differences:

• Less aggressive course: Obsolete acute glomerulonephritis tends to have a less aggressive course compared to rapidly progressive glomerulonephritis.
• Different pathological features: The presence of crescents in > 50% of glomeruli is more characteristic of rapidly progressive glomerulonephritis.

Current Understanding

The current understanding is that obsolete acute glomerulonephritis with lesion of rapidly progressive glomerulonephritis represents a distinct entity within the spectrum of glomerulonephritis. It is essential to differentiate it from rapidly progressive glomerulonephritis, as the treatment and prognosis may vary.

References

• [1] Glomerulonephritis is a heterogeneous group of disorders that can present with acute or chronic kidney injury.
• [3] Rapidly progressive glomerulonephritis is characterized by rapid loss of renal function over a short period.
• [8] Immunopathologic studies have demonstrated that rapidly progressive glomerulonephritis can result from glomerular deposition of anti-GBM antibody, immune complexes, or some undefined mechanism.

Note: The term "obsolete" refers to the fact that this condition was once considered an acute presentation of rapidly progressive glomerulonephritis but has since been reclassified as a distinct entity.

Based on the search results, here are the signs and symptoms associated with obsolete acute glomerulonephritis with lesions of rapidly progressive glomerulonephritis:

• Rapidly progressive renal failure: This condition is characterized by a swift decline in kidney function, often over a few months, accompanied by severe symptoms [9].
• Hematuria: Pink or cola-colored urine from red blood cells in the urine is a common symptom of glomerulonephritis, including rapidly progressive glomerulonephritis [10][12].
• Proteinuria: Foamy or bubbly urine due to excess protein in the urine is another symptom associated with glomerulonephritis [10][12].
• Hypertension: High blood pressure is a common finding in patients with rapidly progressive glomerulonephritis [1][9].
• Azotemia: Elevated levels of urea and creatinine in the blood are indicative of renal failure, which is a hallmark of rapidly progressive glomerulonephritis [1].
• Rapidly progressive glomerulonephritis (RPGN) syndrome: This syndrome is characterized by an accelerated loss of renal function over a short period, unusual urine analysis, and crescent formation in renal biopsies [13].

Additionally, some patients may experience extra-renal signs and symptoms, such as:

• Arthralgias: Joint pain or inflammation
• Skin rash: A skin rash can be present in some cases of rapidly progressive glomerulonephritis
• Pericarditis: Inflammation of the pericardium (the sac surrounding the heart)
• Peripheral neuropathy: Nerve damage affecting the limbs
• Rhinitis: Inflammation of the nasal passages
• Sinusitis: Inflammation of the sinuses

It's worth noting that not all patients with rapidly progressive glomerulonephritis will exhibit these symptoms, and some may experience a more gradual decline in renal function.

Based on the search results, it appears that diagnostic tests for acute glomerulonephritis (AGN) have evolved over time. Here are some relevant findings:

• Serum complement levels: Measuring serum complement levels (C3, C4) and/or activity (CH50) is a practical approach to diagnosis and management of AGN (13).
• Anti-GBM antibody test: A positive result for anti-GBM antibodies can confirm the diagnosis of Goodpasture syndrome, which is a type of AGN (7, 8).
• Kidney biopsy: Renal biopsy is required for definitive diagnosis of IgA nephropathy and other forms of AGN, including rapidly progressive glomerulonephritis (RPGN) (14, 9).

However, it's worth noting that the diagnostic approach has changed over time. For example:

• Primary Glomerulonephritis: In a study from 2012, Primary Glomerulonephritis was the main diagnosis reported in 65.3% of cases, with Immunoglobulin A Nephropathy (IgAN) being the commonest lesion in primary glomerulonephritis (15).
• RPGN: Rapidly progressive glomerulonephritis is a rare and severe form of AGN that can present with full-blown nephritic syndrome or rapidly progressive kidney failure. A kidney biopsy is usually performed at presentation to confirm the diagnosis and rule out other conditions (9).

In summary, while some diagnostic tests for acute glomerulonephritis remain relevant today, others have become obsolete due to advances in medical knowledge and technology.

References:

[13] Table 15-1 presents major causes of acute glomerulonephritis and uses serum complement levels as a tool to focus the differential diagnosis. [7] The diagnosis is confirmed by the detection of circulating anti-GBM antibodies, although their levels do not correlate with disease severity (27). [8] A serum test for anti-GBM antibody was positive. To our knowledge, this is the first report of Goodpasture syndrome in a patient with rapidly progressive glomerulonephritis. [9] Renal biopsy is required for definitive diagnosis of IgA nephropathy and other forms of AGN, including rapidly progressive glomerulonephritis (RPGN). [15] Primary Glomerulonephritis was the main diagnosis reported in 65.3% of cases, with Immunoglobulin A Nephropathy (IgAN) being the commonest lesion in primary glomerulonephritis.

Based on the search results, it appears that there are various treatment options for rapidly progressive glomerulonephritis (RPGN), which is a form of glomerulonephritis characterized by loss of renal function within weeks.

Treatment Options:

• Glucocorticoids: Glucocorticoids such as prednisone are commonly used to treat RPGN. The treatment typically involves a short course of high-dose glucocorticoids followed by a tapering regimen [1, 5].
• Immunosuppressive therapy: Patients with a rapidly progressive, organ-threatening or life-threatening cryoglobulinaemic syndrome, such as RPGN, should be administered immunosuppressive therapy, regardless of the origin of the cryoglobulins. A short course of glucocorticoids combined with rituximab is recommended [7].
• Cyclophosphamide: Cyclophosphamide has been used in combination with glucocorticoids to treat RPGN. However, its use may be limited due to potential side effects [10].
• Azathioprine: Azathioprine can be used as a replacement for cyclophosphamide in maintenance treatment [11].

Other Treatment Considerations:

• Supportive care: The treatment for acute glomerulonephritis is primarily supportive, including diuretics to reduce edema and antihypertensive drugs to reduce blood pressure [6].
• Antibiotics: Administering antibiotics (penicillin or erythromycin) for 10 days can help ensure eradication of the streptococcus if the disease is believed to be acute post-infectious glomerulonephritis [14].

It's essential to note that the treatment approach may vary depending on individual patient factors and the underlying cause of RPGN. A comprehensive treatment plan should be developed in consultation with a healthcare professional.

References:

[1] Matsuo S, et al. Guidelines for the treatment of rapidly progressive glomerulonephritis, second version. Nihon Jinzo Gakkai Shi. 2011;53:509–55. [5] Koyama A, et al. A nationwide survey of rapidly progressive glomerulonephritis in Japan: etiology, prognosis and treatment diversity. Clin Exp Nephrol. 2009;13:633–50. [7] by FB Cortazar · 2023 · Cited by 16 — Current standard-of-care therapy for severe AAV is glucocorticoids combined with either rituximab or cyclophosphamide. [10] by AAA Hasan · 2015 · Cited by 1 — Cyclosporine was added to enhance management of proteinuria. The patient responded well to the combination of steroids, mycophenolyate mofetil and cyclosporine. [11] by G Moroni · 2014 · Cited by 178 — Although some patients requiring dialysis may recover a good renal function, usually the higher the serum cre- atinine at presentation the worse the outcome. [14] Oct 31, 2023 — Administer antibiotics (penicillin or erythromycin) for 10 days to ensure eradication of the streptococcus if the disease is believed to be acute post-infectious glomerulonephritis.

The differential diagnosis of acute glomerulonephritis (GN) with lesions resembling rapidly progressive glomerulonephritis (RPGN) can be challenging. However, based on the search results, here are some possible conditions that may need to be considered:

• Malignant hypertension: This condition can present with severe glomerulonephritis and rapid deterioration of renal function, similar to RPGN [6]. However, it is typically distinguished from RPGN by its more rapid progression and presence of malignant features.
• Crescentic glomerulonephritis: This type of GN is characterized by the presence of glomerular crescents, which can be mistaken for RPGN. However, it tends to have a more gradual onset and may not progress as rapidly [9].
• Goodpasture's disease (GN): This autoimmune disorder can cause RPGN-like symptoms, including rapid progression of renal failure and hematuria. It is often associated with anti-GBM antibodies [10].
• Lupus nephritis: This condition can also present with RPGN-like features, including rapid deterioration of renal function and glomerulonephritis. However, it is typically associated with systemic lupus erythematosus (SLE) symptoms.
• Other forms of GN: Other types of glomerulonephritis, such as IgA nephropathy or membranous nephropathy, can also present with RPGN-like features and may need to be considered in the differential diagnosis.

It's worth noting that the diagnosis of RPGN is often based on a combination of clinical presentation, laboratory findings, and histopathological examination. A thorough evaluation of the patient's medical history, physical examination, and laboratory results is essential to determine the underlying cause of the renal dysfunction [3].

References:

[1] AAA Hasan (2015) - Patients with autosomal dominant polycystic kidney disease (ADPKD) can suffer from the same causes of acute kidney injury as the general population. [3] LA Hebert (2013) - This work is intended to facilitate accurate diagnosis in the individual patient presenting with evidence of glomerular disease. [6] LA Hebert (2013) - Generally, malignant hypertension can be easily distinguished from severe glomerulonephritis, which usually does not present with malignant features. [9] FA Falix (2018) - The few available studies on pediatric RPGN report that it is caused in over 80% by a form of immune-complex-mediated glomerulonephritis, with crescentic GN being the most common type. [10] by F Pesce · 2021 · Cited by 34 — AKI episodes in glomerular disease are usually due to rapidly progressive glomerulonephritis (RPGN), in which the renal function declines over days or weeks.