 purple Henoch-Schonlein |
Henoch - Schonlein (HSP) can be considered a systemic vasculitis with predominant skin involvement that affects mainly children and rarely adults (1.2). The disease is characterized by tissue deposition of immune complexes containing IgA. This makes it very similar to IgA nephropathy to share some aspects with the renal histology. The framework
skin characterized by palpable purpura and / or petechiae is very similar to that found in the course of cryoglobulinemia and hypersensitivity vasculitis.
skin characterized by palpable purpura and / or petechiae is very similar to that found in the course of cryoglobulinemia and hypersensitivity vasculitis.
The HSP has been reported in cases ranging in age from 6 months to 86 years but most patients are children. The average age is 6 years, 75% of patients younger than 8 years and 90% less than 10 years. The more young age seem to be less severe clinical symptoms. In younger children nephritis and abdominal complications are more rare and less severe. On the other hand seems to be moving the disease in adults with more severe symptoms, and nephritis be more frequent. Some authors report a frequency of renal complications in 85% of adults in a 13% with severe renal impairment. Even the possibility of recurrence is more common in adults.
Most patients become ill in the autumn or winter and HSP is often preceded by respiratory tract infections. Many bacteria have been suspected as triggering factors including: b-hemolytic streptococci group A, hepatitis B, herpex virus, parvovirus B19, Coxsackie virus, adenovirus, helicobacter pylori. Even environmental factors and drugs have been viewed with suspicion but no proof certain.
Most patients become ill in the autumn or winter and HSP is often preceded by respiratory tract infections. Many bacteria have been suspected as triggering factors including: b-hemolytic streptococci group A, hepatitis B, herpex virus, parvovirus B19, Coxsackie virus, adenovirus, helicobacter pylori. Even environmental factors and drugs have been viewed with suspicion but no proof certain.
Events Clinics
The clinical manifestations of HSP are shown and are made in the classic tetrad: rash, arthralgia, abdominal pain and renal disease. However, rarely may be affected other organs such as the central nervous system and lung.
The purple skin is the essential criterion for the purpuric lesions may be preceded by urticarial manifestations, sometimes associated with edema, or macular and papular lesions. The classic picture is made up of palpable purpura with lesions of 2-10 mm in diameter affecting the lower limbs symmetrically and / or buttocks.
Arthritis is the second event in the 75% of cases and mainly affects the knees and ankles. Initially it may be highly disabling but resolves spontaneously and does not lead to joint deformities.
bowel involvement is present in 50-75% of patients and is mostly characterized by abdominal colic, vomiting and bleeding of the intestinal tract. The intussusezione is a rare complication (1-5% of cases) but serious. In contrast to the classical localization of the ileo-colic intussusezione idiopathic, in the course of HSP is ileo-ileal in 70% of cases.
nephritis is present in 40% of cases and the main symptom is hematuria. The presence of microscopic hematuria is a constant in the course of the disease but in some cases there will be considerable severity of gross hematuria. In 2 / 3 of patients with renal involvement proteinuria is present but proteinuria alone is rare. The presence of renal involvement is not correlated to the severity of other clinical conditions, in particular not necessarily an important framework to associate a skin is just as important renal disease. It appears that the HSP nephritis represents about 15% of all glomerulopathies' s childhood.
There is a correlation between the severity of renal biopsy and organ. Patients with asymptomatic hematuria alone were mostly focal mesangial glomerulonephritis. The appearance of proteinuria is usually associated with an increased proliferative component while the presence of nephrotic proteinuria is frequently associated the presence of crescent.
The percentage of glomeruli with crescent there seems to be the most important prognostic data for the evolution of renal disease. In a study of 151 children followed children for 18 years showed that: 1
. Among patients with crescent in 50% of glomeruli 37% progressed to complete renal failure and il18% to a chronic renal failure.
2. 86% of children who were facing crescent with renal insufficiency had at least 50% of glomeruli.
3.Il 70% of patients who were cured had few or no crescent in the renal biopsy.
There is a correlation between the severity of renal biopsy and organ. Patients with asymptomatic hematuria alone were mostly focal mesangial glomerulonephritis. The appearance of proteinuria is usually associated with an increased proliferative component while the presence of nephrotic proteinuria is frequently associated the presence of crescent.
The percentage of glomeruli with crescent there seems to be the most important prognostic data for the evolution of renal disease. In a study of 151 children followed children for 18 years showed that: 1
. Among patients with crescent in 50% of glomeruli 37% progressed to complete renal failure and il18% to a chronic renal failure.
2. 86% of children who were facing crescent with renal insufficiency had at least 50% of glomeruli.
3.Il 70% of patients who were cured had few or no crescent in the renal biopsy.
It 'important to remember that some symptoms may precede the purple and thus make the diagnosis more difficult. The arthritis may precede the purple over a week in 25% of cases and may precede intestinal involvement of the purple two weeks in 20% of patients. Hardly nephritis above the main symptom and may even follow the purple and the other symptoms by weeks or months. In fact 75% of patients with nephritis will do it within 4 weeks and 95% within 3 months.
nephritis is the only event that can otherwise become chronic disease tends to resolve within a month.
1 / 3 of patients have relapses but even these tend to resolve within four months. Some authors argue that a large number of relapses predisposes to the development renal disease.
nephritis is the only event that can otherwise become chronic disease tends to resolve within a month.
1 / 3 of patients have relapses but even these tend to resolve within four months. Some authors argue that a large number of relapses predisposes to the development renal disease.
Diagnosis
In children the symptoms are characteristic tetrad of HSP is virtually characteristic idisturbi diseases such as bleeding or sepsis can easily be excluded. In adults should be excluded but other systemic diseases and autoimmune diseases such as hypersensitivity vasculitis or lupus erythematosus sitemi that could cause similar symptoms. Confirmation of the diagnosis of HSP requires the demonstration of tissue deposits of IgA in skin biopsies or in the kidneys. Skin biopsy shows the presence of a leukocytoclastic vasculitis predominantly post-capillary veins. This finding is not specific (as is found in other forms of vasculitis) unless it is accompanied by vascular deposits of IgA that can sometimes be seen even in non-affected skin.
There are also some criteria that help distinguish between HSP and hypersensitivity vasculitis. In one study it was stated that this is a hypersensitivity vasculitis and not an HSP with an accuracy of 74% if NOT have more than two of the following criteria:
1.porpora palpable
2.dolore abdominal
3.Sanguinamento intestinal
4.ematuria
5.Età of onset> 20 years.
2.dolore abdominal
3.Sanguinamento intestinal
4.ematuria
5.Età of onset> 20 years.
Prognosis
Overall, the prognosis is good in most patients. In children, complete recovery occurs in 94% of cases. but, considering the adults, only 89% of patients.
Most events resolve spontaneously even if they may have recurrent episodes of glomerulonephritis which is purple.
The prognosis of renal disease is also good as it seems that the disease is responsible for 3% of cases of renal insufficiency of childhood.
Considering some work on this we can say that in children:
Most events resolve spontaneously even if they may have recurrent episodes of glomerulonephritis which is purple.
The prognosis of renal disease is also good as it seems that the disease is responsible for 3% of cases of renal insufficiency of childhood.
Considering some work on this we can say that in children:
1. After 2 years, half is in complete remission, while 1 / 3 of patients had persistent abnormal urine examination but with normal renal function
2. On 88 children followed for 10 years, most of those with only abnormal urine examination has been in complete remission while some of those with renal failure at presentation progressed to complete renal failure.
3.Su seguti 78 children 17 to 23 years (22%) had deterioration of renal function clinch. In this group there were also seven who had gone into complete remission after 10 years. Yet 36% of 44 pregnancies were complicated by hypertension and proteinuria.
2. On 88 children followed for 10 years, most of those with only abnormal urine examination has been in complete remission while some of those with renal failure at presentation progressed to complete renal failure.
3.Su seguti 78 children 17 to 23 years (22%) had deterioration of renal function clinch. In this group there were also seven who had gone into complete remission after 10 years. Yet 36% of 44 pregnancies were complicated by hypertension and proteinuria.
To explain this evolution in the time it was assumed that during the phase of HSP is you had an irreversible reduction in the number of nephrons that drove the remaining to hyperfiltration and compensatory hypertrophy. After years, these changes would lead to glomerular damage independent of the disease (HSP) that had triggered.
Therapy
So far there are no prospective controlled studies on the use of corticosteroids in HSP although many data suggest their efficacy in the treatment of arthritis and abdominal pain. However, there are no data to suggest their efficacy in the treatment of purpura on duration of the illness or the possibility of preventing recurrence.
Few are the evidence of their efficacy in the treatment of renal complications although in an uncontrolled but prospective study in children with severe nephritis (> 50% crescent of the renal biopsy) of steroid boluses (30 mg / kg / daily for 3 days) have been able to improve the glomerulonephritis and prevent the progression of the disease.
Other studies have shown the efficacy of bolus steroids followed by oral treatment is associated with azathioprine or cyclophosphamide in the treatment of glomerulonephritis. The plasma exchange was effective in the treatment of a few cases with rapidly progressive glomerulonephritis secondary to HSP.
Few are the evidence of their efficacy in the treatment of renal complications although in an uncontrolled but prospective study in children with severe nephritis (> 50% crescent of the renal biopsy) of steroid boluses (30 mg / kg / daily for 3 days) have been able to improve the glomerulonephritis and prevent the progression of the disease.
Other studies have shown the efficacy of bolus steroids followed by oral treatment is associated with azathioprine or cyclophosphamide in the treatment of glomerulonephritis. The plasma exchange was effective in the treatment of a few cases with rapidly progressive glomerulonephritis secondary to HSP.
Bibliography
1) Allen DM et al. Am J Dis Child 1960, 99:833
2) FT Saulsbury et al Medicine 1999, 78:395
2) FT Saulsbury et al Medicine 1999, 78:395
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