Shunt nephritis

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Shunt nephritis
Immune22.gif
Shunt nephritis is caused by the deposition of immune complexes, as shown in this illustration.
Specialty Nephrology

Shunt nephritis is a rare disease of the kidney that can occur in patients being treated for hydrocephalus with a cerebral shunt. It usually results from an infected shunt that produces a long-standing blood infection, particularly by the bacterium Staphylococcus epidermidis . Kidney disease results from an immune response that deposits immune complexes in the kidney. The most common signs and symptoms of the condition are blood and protein in the urine, anemia, and high blood pressure. Diagnosis is based on these findings in the context of characteristic laboratory values. Treatment includes antibiotics and the prompt removal of the infected shunt. Over half of individuals with shunt nephritis recover completely; most of the remainder have some degree of persistent kidney disease.

Contents

Presentation

The clinical presentation of shunt nephritis is variable, but the most common manifestations of shunt nephritis include blood in the urine, protein in the urine, anemia, and high blood pressure. [1] Recurrent fever, enlarged liver and spleen, and a skin rash may also be present. Rarely, the major complaint may be arthritis. [2]

Pathophysiology

Shunt nephritis occurs when a shunt becomes infected with bacteria, most commonly Staphylococcus epidermidis. Bacteria from this infected shunt seed the bloodstream, leading to blood infection (bacteremia). In response to long-standing infection (months to years), the body mounts an immune response that results in deposition of immune complexes in the kidney, leading to nephritis. [1]

Diagnosis

Urinalysis typically demonstrates hematuria and proteinuria. Levels of the complement protein C3 are low, while levels of C-reactive protein and cryoglobulins may be modestly elevated. Blood cultures and cerebrospinal fluid cultures demonstrate Staphylococcus epidermidis , a coagulase-negative species of Staphylococcus . Biopsy of the kidney frequently demonstrates membranoproliferative glomerulonephritis, with deposits of C3, IgM, and IgG. [1]

Treatment

Management is focused on removing the infectious source. The shunt is removed immediately and antibiotics are begun. The infected shunt, typically a ventriculoatrial shunt, may be replaced with a ventriculoperitoneal shunt. [3]

Prognosis

In one review, over half of individuals with shunt nephritis made a complete recovery. An additional 40% of individuals had persistent urine abnormalities or end-stage renal disease. Death occurred in 9%. [1]

Epidemiology

Shunt nephritis is a rare condition affecting males and females of all ages. It occurs in approximately 0.7-2.3% of patients with shunt infections. Approximately 12% of ventriculoatrial shunts become infected, with Staphylococcus epidermidis being the infectious agent in 75% of cases. [1]

History

Shunt nephritis was first described by Black in 1965. [4] Early cases and most cases since then are associated with infections of shunts that connect the ventricular system of the brain to the atria of the heart (ventriculoatrial shunts). Less commonly, shunt nephritis has been reported to arise from infections of shunts connecting the ventricular system to the abdominal cavity (ventriculoperitoneal shunts). [5]

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<i>Staphylococcus epidermidis</i> Species of bacterium

Staphylococcus epidermidis is a Gram-positive bacterium, and one of over 40 species belonging to the genus Staphylococcus. It is part of the normal human flora, typically the skin flora, and less commonly the mucosal flora. It is a facultative anaerobic bacteria. Although S. epidermidis is not usually pathogenic, patients with compromised immune systems are at risk of developing infection. These infections are generally hospital-acquired. S. epidermidis is a particular concern for people with catheters or other surgical implants because it is known to form biofilms that grow on these devices. Being part of the normal skin flora, S. epidermidis is a frequent contaminant of specimens sent to the diagnostic laboratory.

Interstitial nephritis Medical condition

Interstitial nephritis, also known as tubulointerstitial nephritis, is inflammation of the area of the kidney known as the renal interstitium, which consists of a collection of cells, extracellular matrix, and fluid surrounding the renal tubules. In addition to providing a scaffolding support for the tubular architecture, the interstitium has been shown to participate in the fluid and electrolyte exchange as well as endocrine functions of the kidney.

Acute proliferative glomerulonephritis Medical condition

Acute proliferative glomerulonephritis is a disorder of the small blood vessels of the kidney. It is a common complication of bacterial infections, typically skin infection by Streptococcus bacteria types 12, 4 and 1 (impetigo) but also after streptococcal pharyngitis, for which it is also known as postinfectious glomerulonephritis (PIGN) or poststreptococcal glomerulonephritis (PSGN). It can be a risk factor for future albuminuria. In adults, the signs and symptoms of infection may still be present at the time when the kidney problems develop, and the terms infection-related glomerulonephritis or bacterial infection-related glomerulonephritis are also used. Acute glomerulonephritis resulted in 19,000 deaths in 2013, down from 24,000 deaths in 1990 worldwide.

Cerebral shunt Surgical implant to treat hydrocephalus

Cerebral shunts are commonly used to treat hydrocephalus, the swelling of the brain due to excess buildup of cerebrospinal fluid (CSF). If left unchecked, the cerebrospinal fluid can build up leading to an increase in intracranial pressure (ICP) which can lead to intracranial hematoma, cerebral edema, crushed brain tissue or herniation. The cerebral shunt can be used to alleviate or prevent these problems in patients who suffer from hydrocephalus or other related diseases.

Lupus Human autoimmune disease

Lupus, technically known as systemic lupus erythematosus (SLE), is an autoimmune disease in which the body's immune system mistakenly attacks healthy tissue in many parts of the body. Symptoms vary between people and may be mild to severe. Common symptoms include painful and swollen joints, fever, chest pain, hair loss, mouth ulcers, swollen lymph nodes, feeling tired, and a red rash which is most commonly on the face. Often there are periods of illness, called flares, and periods of remission during which there are few symptoms.

Diffuse proliferative glomerulonephritis (DPGN) is a type of glomerulonephritis that is the most serious form of renal lesions in SLE and is also the most common, occurring in 35% to 60% of patients. Most of the glomeruli show endothelial and mesangial proliferation, affecting the entire glomerulus, leading to diffuse hypercellularity of the glomeruli, producing in some cases epithelial crescents that fill Bowman's space. When extensive, immune complexes create an overall thickening of the capillary wall, resembling rigid "wire loops" on routine light microscopy. Electron microscopy reveals electron-dense subendothelial immune complexes. Immune complexes can be visualized by staining with fluorescent antibodies directed against immunoglobulins or complement, resulting in a granular fluorescent staining pattern. In due course, glomerular injury gives rise to scarring (glomerulosclerosis). Most of these patients have hematuria with moderate to severe proteinuria, hypertension, and renal insufficiency.

References

  1. 1 2 3 4 5 Haffner D, Schindera F, Aschoff A, Matthias S, Waldherr R, Schärer K (June 1997). "The clinical spectrum of shunt nephritis". Nephrol. Dial. Transplant. 12 (6): 1143–8. doi: 10.1093/ndt/12.6.1143 . PMID   9198042.
  2. Legoupil N, Ronco P, Berenbaum F (May 2003). "Arthritis-related shunt nephritis in an adult". Rheumatology (Oxford). 42 (5): 698–9. doi: 10.1093/rheumatology/keg151 . PMID   12709554.
  3. Noiri E, Kuwata S, Nosaka K, et al. (April 1993). "Shunt nephritis: efficacy of an antibiotic trial for clinical diagnosis". Intern. Med. 32 (4): 291–4. doi: 10.2169/internalmedicine.32.291 . PMID   8358118.
  4. Black JA, Challacombe DN, Ockenden BG (November 1965). "Nephrotic syndrome associated with bacteraemia after shunt operations for hydrocephalus". Lancet. 2 (7419): 921–4. doi:10.1016/S0140-6736(65)92901-6. PMID   4165274.
  5. Noe HN, Roy S (May 1981). "Shunt nephritis". J. Urol. 125 (5): 731–3. doi:10.1016/S0022-5347(17)55183-6. PMID   7230350.