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DEFINITIONIs a clinical syndrome caused by systemic vasculitis of small blood vessels are characterized by specific lesions of nontrombositopenik purpura, arthritis or arthralgia, abdominal pain or gastrointestinal bleeding, and sometimes nephritis or haematuria. Another name of this disease is anaphylactoid purpura, allergic purpura and allergic vasculitis.
EPIDEMIOLOGY
The disease is mainly found in children aged 2-15 years (school age children) with a peak at age 4-7 years. There is more on boys than girls (1.5: 1)
ETIOLOGY
Until now the cause of this disease is unknown. Allegedly several factors play a role, among other genetic factors, upper respiratory tract infection, food, insect bites, exposure to cold, immunization (vaccine varicella, rubella, rubeolla, hepatitis A and B, paratyphoid A and B, typhoid, cholera) and antibiotics (ampisillin, erythromycin, quinine, penicillin, quinidin, quinine). Infection can be caused by bacteria (species of Haemophilus, Mycoplasma, Parainfluenzae, Legionella, Yersinia, Shigella and Salmonella) or viruses (adenovirus, varicella, parvovirus, Epstein-Barr virus). Vasculitis can also develop after therapy antireumatik, including the use of methotrexate and anti-TNF agent (Tumour Necrosis Factor). However, IgA clearly has an important role, characterized by increased concentrations of serum IgA, IgA immune complexes and deposit in blood vessel walls and renal mesangium. HSP is a disorder that is almost always associated with abnormalities in IgA1 than IgA2
PATHOPHYSIOLOGY
From biopsies of lesions on the skin or kidneys, there are known deposits of immune complexes containing IgA. Note also the existence of alternative pathway complement activation. Deposits of immune complexes and complement activation resulting in activation of inflammatory mediators including vascular prostaglandins such as prostacyclin, causing inflammation in small blood vessels in the skin, kidneys, joints and abdomen and occurs in the skin purpura, nephritis, arthritis and gastrointestinal bleeding.
CLINICAL
HSP usually appears with a rash triad of purpura on the lower extremities, abdominal pain or kidney disorders and arthritis. But the triad is not always there, so that often lead to incorrect diagnosis. Clinical symptoms early - the early form of eritomatosa macular rash on the skin of the lower limb which continues to be a symmetric palpable purpura without thrombocytopenia. The rash was initially limited to the skin but usually malleolus will then be extended to the dorsal surface of the legs, buttocks and outer sleeve. Within 12-24 hours macular purpuric lesions will turn into a dark red and has a diameter of 0.5 to 2 cm. Lesions may coalesce into larger plaques that resemble echimosis which can then be ulcerated.
Purpura mainly found on the skin that are often exposed to pressure (pressure-bearing surfaces). This skin disorder is found at 100% of cases and represents 50% of complaints patients at the time of treatment. Skin disorders can also be found on the face and body. Abnormalities of the skin can be itchy. In the form that is not classical, the existing skin disorders can form vesicles to resemble erythema multiform. Acute disorders of the skin can last several weeks and disappear, but can also be recurrent. Scrotal Edema can also occur and the symptoms are similar to testicular torsion. Prodromal symptoms can include fever with a temperature of not more than 38 ° C, headache and anorexia.
HSP usually appears with a rash triad of purpura on the lower extremities, abdominal pain or kidney disorders and arthritis. But the triad is not always there, so that often lead to incorrect diagnosis.
The disease is mainly found in children aged 2-15 years (school age children) with a peak at age 4-7 years. There is more on boys than girls (1.5: 1)
ETIOLOGY
Until now the cause of this disease is unknown. Allegedly several factors play a role, among other genetic factors, upper respiratory tract infection, food, insect bites, exposure to cold, immunization (vaccine varicella, rubella, rubeolla, hepatitis A and B, paratyphoid A and B, typhoid, cholera) and antibiotics (ampisillin, erythromycin, quinine, penicillin, quinidin, quinine). Infection can be caused by bacteria (species of Haemophilus, Mycoplasma, Parainfluenzae, Legionella, Yersinia, Shigella and Salmonella) or viruses (adenovirus, varicella, parvovirus, Epstein-Barr virus). Vasculitis can also develop after therapy antireumatik, including the use of methotrexate and anti-TNF agent (Tumour Necrosis Factor). However, IgA clearly has an important role, characterized by increased concentrations of serum IgA, IgA immune complexes and deposit in blood vessel walls and renal mesangium. HSP is a disorder that is almost always associated with abnormalities in IgA1 than IgA2
PATHOPHYSIOLOGY
From biopsies of lesions on the skin or kidneys, there are known deposits of immune complexes containing IgA. Note also the existence of alternative pathway complement activation. Deposits of immune complexes and complement activation resulting in activation of inflammatory mediators including vascular prostaglandins such as prostacyclin, causing inflammation in small blood vessels in the skin, kidneys, joints and abdomen and occurs in the skin purpura, nephritis, arthritis and gastrointestinal bleeding.
CLINICAL
HSP usually appears with a rash triad of purpura on the lower extremities, abdominal pain or kidney disorders and arthritis. But the triad is not always there, so that often lead to incorrect diagnosis. Clinical symptoms early - the early form of eritomatosa macular rash on the skin of the lower limb which continues to be a symmetric palpable purpura without thrombocytopenia. The rash was initially limited to the skin but usually malleolus will then be extended to the dorsal surface of the legs, buttocks and outer sleeve. Within 12-24 hours macular purpuric lesions will turn into a dark red and has a diameter of 0.5 to 2 cm. Lesions may coalesce into larger plaques that resemble echimosis which can then be ulcerated.
Purpura mainly found on the skin that are often exposed to pressure (pressure-bearing surfaces). This skin disorder is found at 100% of cases and represents 50% of complaints patients at the time of treatment. Skin disorders can also be found on the face and body. Abnormalities of the skin can be itchy. In the form that is not classical, the existing skin disorders can form vesicles to resemble erythema multiform. Acute disorders of the skin can last several weeks and disappear, but can also be recurrent. Scrotal Edema can also occur and the symptoms are similar to testicular torsion. Prodromal symptoms can include fever with a temperature of not more than 38 ° C, headache and anorexia.
HSP usually appears with a rash triad of purpura on the lower extremities, abdominal pain or kidney disorders and arthritis. But the triad is not always there, so that often lead to incorrect diagnosis.
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| HENOCH-SCHÖNLEIN PURPURA |
Clinical symptoms early - the early form of eritomatosa macular rash on the skin of the lower limb which continues to be a symmetric palpable purpura without thrombocytopenia. The rash was initially limited to the skin but usually malleolus will then be extended to the dorsal surface of the legs, buttocks and outer sleeve. Within 12-24 hours macular purpuric lesions will turn into a dark red and has a diameter of 0.5 to 2 cm. Lesions may coalesce into larger plaques that resemble echimosis which can then be ulcerated.
Purpura mainly found on the skin that are often exposed to pressure (pressure-bearing surfaces). This skin disorder is found at 100% represents 50% of cases and complaints of patients at the time of treatment. Skin disorders can also be found on the face and body. Abnormalities of the skin can be itchy. In the form that is not classical, the existing skin disorders can form vesicles to resemble erythema multiform. Acute disorders of the skin can last several weeks and disappear, but can also be recurrent. Scrotal Edema can also occur and the symptoms are similar to testicular torsion. Prodromal symptoms can include fever with a temperature of not more than 38 ° C, headache and anorexia.
In children aged less than 2 years, the clinical picture is dominated oelh sinfulness scalp edema, periorbital, hands and feet. This picture is called AHEI (Acute Hemorrhagic Edema of Infancy).
In addition to purpura, also found that symptoms of arthralgia and arthritis tend to be migrants and about the joints of the lower extremities such as knees and ankles, but can also on the wrist, elbow and finger joints in the hand. This disorder arises first (1-2 days) of skin disorders. Affected joints may become swollen, painful and sore when actuated, usually without effusion, redness or heat. Abnormalities teutama periartrikular and temporary, may also recurrent during active disease but does not cause permanent deformity.
In this disease can be found in the form of abdominal discomfort abdominal pain or gastrointestinal bleeding. Abdominal complaints usually arise after the onset of disorders of the skin (1-4 weeks after onset). The organ most frequently involved are the duodenum and small intestine. Abdominal pain can be severe abdominal colic, location in periumbilikal and accompanied by nausea, vomiting, vomiting blood and even sometimes there is a bowel perforation and intussusception is more common than ileoileal ileokolonal. Intussusception or perforation caused by vasculitis that causes the intestinal wall edema and submucosal and intramural hemorrhage. Sometimes it can also occur with a perforated bowel infarction or not.
Moreover, it can also be found renal abnormalities, including hematuria, proteinuria (<2g / d), nephrotic syndrome (proteinuria> 40mg/m2 / hour) or nephritis. Diseases of the kidney is also usually appear 1 month after the onset of skin rash. The existence of a persistent skin disorder until 2-3 months, usually associated with nephropathy or severe kidney disease. Increased risk of nephritis at the age of 7 years, persistent purpura lesions, severe abdominal complaints funds decreased factor XIII activity. Renal impairment is usually mild, although some will become chronic. Often the severity of nephritis is not related to the severity of other symptoms of HSP. In patients with HSP may arise of edema. Edema does not depend on the degree of proteinuria but rather on the degree of vasculitis that occur. However, edema is indeed associated with the incidence of proteinuria in patients.
Sometimes, HSP may be accompanied by symptoms of central nervous system disorders, especially headaches. HSP can be found on the existence of cerebral vasculitis. In some rare cases, HSP allegedly can cause serious disorders such as seizures, paresis, or coma. The symptoms of other neurological disorders that may arise, among others, changes in the level of awareness, apathy, somnolence, hyperactivity, irritability, emotional instability, seizures (partial, complex partial, generalized, status epilepticus), and focal neurologic deficits (aphasia, ataxia, khorea, hemiparese, paraparese, quadraparese. It can also happen poliradikuloneuropathy (Guillain-Barré syndrome) and mononeuropathy (facial nerve, femoral, ulnar).
Liver and gall bladder symptoms may also be involved with hepatomegaly, hydrops of the gallbladder, cholecystitis. All this can lead to complaints of abdominal pain in patients. Acute appendicitis has also been reported in patients with HSP.
Symptoms - other symptoms that have been reported but are rare among other miokardia vasculitis, pulmonary vasculitis that causes pulmonary hemorrhage bilaterally, ureteritis stenosis, penile edema, orchitis, priapism, intracranial hemorrhage, subperiosteal orbital hematoma bilateral adrenal hematoma and acute pancreatitis.
INVESTIGATION SUPPORTING
In laboratory tests there have been no specific abnormalities. Normal or elevated platelet counts, distinguishing purpura caused by thrombocytopenia. Can occur moderate leukocytosis and anemia normochromic, usually associated with gastrointestinal bleeding. Usually there are also eosinophilia. Erythrocyte sedimentation rate can be increased or normal. Levels of complement such as C1q, C3 and C4 may be normal or decreased. Examination of IgA levels in the blood may be increased, so did the lymphocytes that contain IgA. Urinalysis may show hematuria, proteinuria and decreased creatinine clearance marks start of kidney damage or because of dehydration, as well as blood in the stool can be found. ANA and RF examination is usually negative, factor VII and XIII can be decreased.
Biopsy of skin lesion showed leukocytoclastic vasculitis. Immunofluorescence showed deposits of IgA and complement in blood vessel walls. On radiological examination can be found a marked decrease in intestinal motility with intestinal lumen dilation or intussusception by barium examination. Sometimes the barium can also correct the intussusception
DIAGNOSIS
Diagnosis is confirmed by more specific clinical symptoms rather than with the aid of investigation. Symptoms that may lead to the diagnosis of HSP is purpurik rash on the skin especially in the buttocks and lower extremities with one or more of the following symptoms: abdominal pain or gastrointestinal bleeding, arthralgia or arthritis, and hematuria or nephritis.
Differential diagnosis of HSP based on symptoms that may arise, among others, acute abdomen, due to meningococcal meningitis, SLE, bacterial endocarditis, ITP, rheumatic fever, Rocky Mountain spotted fever, allergic reaction to medication - drugs, IgA nephropathy, rheumatoid arthritis
TREATMENT
There is no definitive treatment in patients with HSP. Treatment is supportive and symptomatic, including maintenance of hydration, nutrition, electrolyte balance and overcome the pain with analgesics. For mild arthritis complaints and fever can be used NSAIDs such as ibuprofen. Dose ibuprofen can be given is 10mg/kgweight/6 hours. Edema can be treated with leg elevation. As long as there are complaints of vomiting and abdominal pain, diet is given in the form of soft food. The use of acetyl salicylic acid should be avoided, because it can lead to impaired platelet function is petechiae and gastrointestinal bleeding. When there are symptoms of an acute abdomen, performed the operation. If there is a progressive kidney disorder can be given in combination with immunosuppressant corticosteroids. IV methylprednisolone can prevent worsening of kidney disease when administered early. The dose can be used is methylprednisolone 250-750 mg / day IV for 3-7 days in combination with cyclophosphamide 100-200 mg / hr for severe acute phase of HSP. Continued with corticosteroids (prednisone 100-200 mg orally) and cyclophosphamide hose daily 100-200 mg / hr for 30-75 days before cyclophosphamide was stopped immediately dantappering-off steroids for up to 6 months.
Prednisone therapy can be administered at a dose of 1-2 mg / kgweight / day orally, divided into 3-4 doses for 5-7 days. Corticosteroids given in a state of disease with very severe symptoms, arthritis, vasculitis of the CNS manifestations, lung and testis, severe abdominal pain, gastrointestinal bleeding, edema and persistent nephrotic syndrome. Giving early in the acute phase may prevent bleeding, obstruction, intussusception and gastrointestinal perforation.
Purpura mainly found on the skin that are often exposed to pressure (pressure-bearing surfaces). This skin disorder is found at 100% represents 50% of cases and complaints of patients at the time of treatment. Skin disorders can also be found on the face and body. Abnormalities of the skin can be itchy. In the form that is not classical, the existing skin disorders can form vesicles to resemble erythema multiform. Acute disorders of the skin can last several weeks and disappear, but can also be recurrent. Scrotal Edema can also occur and the symptoms are similar to testicular torsion. Prodromal symptoms can include fever with a temperature of not more than 38 ° C, headache and anorexia.
In children aged less than 2 years, the clinical picture is dominated oelh sinfulness scalp edema, periorbital, hands and feet. This picture is called AHEI (Acute Hemorrhagic Edema of Infancy).
In addition to purpura, also found that symptoms of arthralgia and arthritis tend to be migrants and about the joints of the lower extremities such as knees and ankles, but can also on the wrist, elbow and finger joints in the hand. This disorder arises first (1-2 days) of skin disorders. Affected joints may become swollen, painful and sore when actuated, usually without effusion, redness or heat. Abnormalities teutama periartrikular and temporary, may also recurrent during active disease but does not cause permanent deformity.
In this disease can be found in the form of abdominal discomfort abdominal pain or gastrointestinal bleeding. Abdominal complaints usually arise after the onset of disorders of the skin (1-4 weeks after onset). The organ most frequently involved are the duodenum and small intestine. Abdominal pain can be severe abdominal colic, location in periumbilikal and accompanied by nausea, vomiting, vomiting blood and even sometimes there is a bowel perforation and intussusception is more common than ileoileal ileokolonal. Intussusception or perforation caused by vasculitis that causes the intestinal wall edema and submucosal and intramural hemorrhage. Sometimes it can also occur with a perforated bowel infarction or not.
Moreover, it can also be found renal abnormalities, including hematuria, proteinuria (<2g / d), nephrotic syndrome (proteinuria> 40mg/m2 / hour) or nephritis. Diseases of the kidney is also usually appear 1 month after the onset of skin rash. The existence of a persistent skin disorder until 2-3 months, usually associated with nephropathy or severe kidney disease. Increased risk of nephritis at the age of 7 years, persistent purpura lesions, severe abdominal complaints funds decreased factor XIII activity. Renal impairment is usually mild, although some will become chronic. Often the severity of nephritis is not related to the severity of other symptoms of HSP. In patients with HSP may arise of edema. Edema does not depend on the degree of proteinuria but rather on the degree of vasculitis that occur. However, edema is indeed associated with the incidence of proteinuria in patients.
Sometimes, HSP may be accompanied by symptoms of central nervous system disorders, especially headaches. HSP can be found on the existence of cerebral vasculitis. In some rare cases, HSP allegedly can cause serious disorders such as seizures, paresis, or coma. The symptoms of other neurological disorders that may arise, among others, changes in the level of awareness, apathy, somnolence, hyperactivity, irritability, emotional instability, seizures (partial, complex partial, generalized, status epilepticus), and focal neurologic deficits (aphasia, ataxia, khorea, hemiparese, paraparese, quadraparese. It can also happen poliradikuloneuropathy (Guillain-Barré syndrome) and mononeuropathy (facial nerve, femoral, ulnar).
Liver and gall bladder symptoms may also be involved with hepatomegaly, hydrops of the gallbladder, cholecystitis. All this can lead to complaints of abdominal pain in patients. Acute appendicitis has also been reported in patients with HSP.
Symptoms - other symptoms that have been reported but are rare among other miokardia vasculitis, pulmonary vasculitis that causes pulmonary hemorrhage bilaterally, ureteritis stenosis, penile edema, orchitis, priapism, intracranial hemorrhage, subperiosteal orbital hematoma bilateral adrenal hematoma and acute pancreatitis.
INVESTIGATION SUPPORTING
In laboratory tests there have been no specific abnormalities. Normal or elevated platelet counts, distinguishing purpura caused by thrombocytopenia. Can occur moderate leukocytosis and anemia normochromic, usually associated with gastrointestinal bleeding. Usually there are also eosinophilia. Erythrocyte sedimentation rate can be increased or normal. Levels of complement such as C1q, C3 and C4 may be normal or decreased. Examination of IgA levels in the blood may be increased, so did the lymphocytes that contain IgA. Urinalysis may show hematuria, proteinuria and decreased creatinine clearance marks start of kidney damage or because of dehydration, as well as blood in the stool can be found. ANA and RF examination is usually negative, factor VII and XIII can be decreased.
Biopsy of skin lesion showed leukocytoclastic vasculitis. Immunofluorescence showed deposits of IgA and complement in blood vessel walls. On radiological examination can be found a marked decrease in intestinal motility with intestinal lumen dilation or intussusception by barium examination. Sometimes the barium can also correct the intussusception
DIAGNOSIS
Diagnosis is confirmed by more specific clinical symptoms rather than with the aid of investigation. Symptoms that may lead to the diagnosis of HSP is purpurik rash on the skin especially in the buttocks and lower extremities with one or more of the following symptoms: abdominal pain or gastrointestinal bleeding, arthralgia or arthritis, and hematuria or nephritis.
Differential diagnosis of HSP based on symptoms that may arise, among others, acute abdomen, due to meningococcal meningitis, SLE, bacterial endocarditis, ITP, rheumatic fever, Rocky Mountain spotted fever, allergic reaction to medication - drugs, IgA nephropathy, rheumatoid arthritis
TREATMENT
There is no definitive treatment in patients with HSP. Treatment is supportive and symptomatic, including maintenance of hydration, nutrition, electrolyte balance and overcome the pain with analgesics. For mild arthritis complaints and fever can be used NSAIDs such as ibuprofen. Dose ibuprofen can be given is 10mg/kgweight/6 hours. Edema can be treated with leg elevation. As long as there are complaints of vomiting and abdominal pain, diet is given in the form of soft food. The use of acetyl salicylic acid should be avoided, because it can lead to impaired platelet function is petechiae and gastrointestinal bleeding. When there are symptoms of an acute abdomen, performed the operation. If there is a progressive kidney disorder can be given in combination with immunosuppressant corticosteroids. IV methylprednisolone can prevent worsening of kidney disease when administered early. The dose can be used is methylprednisolone 250-750 mg / day IV for 3-7 days in combination with cyclophosphamide 100-200 mg / hr for severe acute phase of HSP. Continued with corticosteroids (prednisone 100-200 mg orally) and cyclophosphamide hose daily 100-200 mg / hr for 30-75 days before cyclophosphamide was stopped immediately dantappering-off steroids for up to 6 months.
Prednisone therapy can be administered at a dose of 1-2 mg / kgweight / day orally, divided into 3-4 doses for 5-7 days. Corticosteroids given in a state of disease with very severe symptoms, arthritis, vasculitis of the CNS manifestations, lung and testis, severe abdominal pain, gastrointestinal bleeding, edema and persistent nephrotic syndrome. Giving early in the acute phase may prevent bleeding, obstruction, intussusception and gastrointestinal perforation.
