Allergic capillary inflammatory disease
Acute intussusception, and often in the ileum position is the most serious complication of observed gastrointestinal damage.
The first time Heberden (1801) has reported a 5-year-old patient with abdominal pain, vomiting, black stools, joint pain, nodular haemorrhage in the legs and blood in the urine. Willan then described the same case with the title "purpura urticans".
In 1837 Schùnlein described a condition called "peliosis rheumatica" with typical manifestations of joint pain and haemorrhage. 37 years later (1874) Schùnlein's former student Henoch described the new syndrome of haemorrhage, severe abdominal pain, black stools in addition to similar manifestations: rheumatism in the joints of the wrists, fingers, neck. foot.
The years 1895-1914 Osler published in England many articles on the manifestations of allergic capillary inflammation related to allergic phenomena. After that Frank proposal was widely used in the US, European and Japanese authors prefer to use the name of the person who found out "Schửnlein-Henoch syndrome".
In 1969, Berger discovered that the interstitial deposition of IgA complexes in primary IgA nephropathy resembled that of allergic capillary nephritis. In 1980, Conley used a monoclonal antibody technique to detect interstitial deposition in Berger disease and mainly subclass IgA1. Two years later, Sevenson reported the simultaneous presence of IgA and C3 in the skin, intestinal vessels and intervascular regions in an adult with allergic capillary inflammation.
Allergic capillaries appear at all ages, but mainly in children 2-16 years old. The child / adult ratio varies from 16-22.
The disease is more common in men than women, the adult sex ratio is the same as in children: male / female ratio = 1.5 / 1. Some authors report equal proportions between men and women, but according to a Japanese study, women dominate.
May occur in months of the year, but occurs more frequently in winter-spring.
The disease is found in races, in some countries such as England, France, Finland, Japan, Singapore, Taiwan, the number of patients is much more than North America, Africa. In South America, the number of patients with glomerulonephritis caused by allergic capillaries is rarer, while the proportion of patients with glomerulonephritis in this region is very high. Allergic capillary inflammation as well as primary IgA nephropathy in blacks is very rare. The above differences can be attributed to both environment and race.
The cause of the allergic capillary inflammation is unknown, usually onset after a respiratory infection several weeks before the onset of illness. Detection of streptococci in the throat and an increase in streptolysin O ratio contribute to the pathogenesis of streptococci. In addition, some other bacteria are also suspected to be the cause such as staphylococci, tuberculosis bacilli, varicella, adenovirus, fungus ... Some patients after taking drugs, vaccination, insect bites.
Mechanism of pathogenesis
Through some experimental studies on animal models and human observations, immune complexes may be the main mechanism in the expression of small vasculitis, but the localization of immune complexes Circulating fluids at particular vascular sites of the disease is still speculative. Detection of immune complex deposition containing IgA, C3, less deposition of IgG and properdin in the interstitial regions of the skin capillaries, the jejunum of the children with nephritis due to allergic capillary inflammation associated with organs It is observed that serum IgA levels increase during the acute phase of the disease, these results contribute to the explanation of the etiology of the immune system.
Purpura haemorrhagic, an allergic capillary-like disease, was observed in horses immediately following an upper respiratory tract infection caused by Streptococcus equip. This disease includes a skin haemorrhage with Leucocytoclastic vasculitis, often glomerulonephritis, elevated serum IgA and circulating PHMD that includes Streptococcus antigens and specific IgAs.
Immune complexes contain IgA
In 1971 Trygstad and Stiehm observed an increase in serum IgA-related subclasses of IgA1 in 50% of their child patients. They also observed a clinical similarity between allergic capillary syndrome and experimental glomerulonephritis, including primary IgA-induced kidney disease, which led to the hypothesis that Allergic capillary inflammation appears to be a disease involving the deposition of immune complexes containing IgA 1 in blood vessels and in the glomeruli.
Allergic capillaries are a systemic disease, the first symptom to appear after an upper respiratory tract infection with the following manifestations: fatigue, low-grade fever, gastrointestinal disturbances, followed by a specific rash and symptoms. Other clinical.
Haemorrhage is the first symptom seen in more than 50% of advanced cases.
Position: face stretched limbs, around ankles inside and outside, thighs, buttocks, arms, forearms; Uncommon in the body, sometimes in the ears, ear canal, nose, external genitals.
Properties: non-itchy; lesions are purulent-like, nodular purpura, usually a ridge above the skin surface (due to infiltration), possibly with urticaria, bullous or bruising, and necrotic erythema; lesions affected by standing (increased by standing); oedema can be detected: oedema soft, concave, usually localized in the scalp, around the fovea, ears, back of arms, instep, heels, sometimes on the genitals, oedema is common in young children; lesions are symmetrical.
Joint manifestations are seen in 75% of cases
Location: joints adjacent to the site of purpura: ankles, knees, elbows, rarely in wrists and hands. The shoulders, toes, and spine can sometimes be affected.
Properties: joint pain, moderate arthritis, limited movement; lesions are usually symmetrical; oedema around joints, sometimes coordinated tendon pain; joint damage that is cured within hours or days, may recur; does not deform joints; For visible muscle damage, a muscle biopsy can reveal necrotic lesions on a motor artery.
Digestion occurs in 37-66% of cases, sometimes as the onset of the disease
Less severe, persistent abdominal pain around the navel, dominant when pressed, possibly diffuse or localized epigastric pain, combined with nausea and vomiting. Pain lasts a few hours or days, or recurs.
Gastrointestinal bleeding manifests itself in vomiting of blood, black or bloody stools, accompanied by severe abdominal pain.
Intestinal and often in the ileum-ileum is the most serious complication of gastrointestinal damage observed in 5% of cases.
Bowel obstruction, infarction, or perforation of the colon may occur.
Possible acute pancreatitis.
Kidney damage is seen in 25-50% in the acute phase
General or microscopic haematuria, proteinuria, prolonged proteinuria is often combined with microscopic haematuria, sometimes leukaemia without infection, appearing after 1 to several weeks or later a few Other manifestations:
Nephrotic syndrome does not merely present: proteinuria> 3g / 24h in adults and> 40mg / m2 body / h in children; coordinate with blood albumin <30g / l; signs are not simply manifested by increased blood pressure or renal failure.
Acute nephritis syndrome may also be present (identified by the presence of haematuria and proteinuria <3g / 24h in adults, <40mg / m2 / h in children) and may or may not have renal failure and increase blood pressure combination).
Some cases of children have a syndrome of rapidly progressive glomerulonephritis (unstable plural proteinuria, haematuria and essentially rapid deterioration of kidney function). Patients with this syndrome have a very poor prognosis.
Chronic kidney failure is a sign of the disease.
Symptoms in other organs are more rare
Testicular inflammation: painful and swollen testicles that usually clear up on their own after a few days.
Heart: myocarditis, pericarditis; Myocardial infarction on a normal coronary artery is associated with minor vasculitis of the heart muscle; arrhythmia; Congestive heart failure due to allergic capillary inflammation.
Lungs: Haemorrhage in the alveoli is a very rare complication in allergic capillary inflammation, but very severe. This sign is never the first symptom of the disease; pleural effusion and haemorrhage.
CNS: can be just headache or behavioural disorder; In more severe cases, meningeal bleeding, convulsions or coma.
Eye: Retinal vasculitis with fundus haemorrhage has been observed in some patients.
Implementing the quadrants
Basing on clinical is mainly, based on complete and typical symptoms such as vascular purpura, joint pain, gastrointestinal manifestations, kidney.
In 1990, the American Society of Rheumatology (ARA) gave 4 diagnostic criteria for allergic capillary inflammation (diagnosis of allergic capillary inflammation if the patient has 2 or more criteria).
(1) Haemorrhagic purpura.
(2) Age <20 at the onset of illness.
(3) Diffuse abdominal pain, increased after meals, frequent bloody diarrheal.
(4) Leucocytoclastic vasculitis picture on skin biopsy.
Purpura: Differentiated from haemorrhage caused by infection: sepsis caused by brain tissue; thrombocytopenic bleeding: decreased platelet count; Systemic lupus erythematosus: if the rash is associated with joint pain; inflammation of the node around the artery.
Arthritis: differentiated from Kawasaki disease, the main clinical manifestation is mucosal rash combined with lymph nodes in many places.
Abdominal manifestations: this should be distinguished from surgical emergencies and sometimes a probe opening is necessary in order not to progress to intussusception or peritonitis.
Kidney: can be misdiagnosed with streptococcal nephritis in case of increased ratio of Streptolysin O and streptococcal is found in the larynx.
Progression and prognosis
Skin damage usually lasts 2-3 weeks, then fades and may recur a few new waves, successive waves lasting about 3 months or even longer since the onset of illness.
Joint symptoms appear transient and go away with no sequelae.
Initial symptoms and sometimes dangerous complications such as intussusception, intestinal obstruction, perforation of the gastrointestinal tract, colon dilatation, in cases of emergency surgery.
Most are completely cured, a few will progress to chronic kidney failure, which is the last stage of any type of kidney damage, so the severity of the kidney damage is a predictor of the disease's prognosis. The general renal manifestations of clinical allergic capillary inflammation are:
Red blood cells or proteinuria is more common than both, of which 20% have gross haematuria.
Some cases manifest acute nephritis with symptoms: oliguria, oedema, hypertension, non-protein nitrogen such as urea and creatinine in the blood increased.
In about 10% of cases, kidney damage with nephrotic syndrome is a sign of unfavourable progression. Patients with a severe prognosis (risk of progressing to chronic renal failure: Nephrotic syndrome or clinical proteinuria predominantly 1g / 24h; Renal function rapidly declines from the start; Age > 50, increased blood pressure; Microtidal proliferation of cells both inside and outside the vessel with sickle-shaped deformation attached).
Some treatment problems
There is no specific therapy, mainly symptomatic treatment, treatment options include:
Rest for 1-2 months, snacks (divided meals, restricting fiber), especially in cases with symptoms in the digestive system.
Take nonsteroidal anti-inflammatory and pain relievers
Analgesics (paracetamol and derivatives) used in the case of joint pain, muscle pain, fever. Nonsteroidal anti-inflammatory drugs are used to treat joint pain that often do not respond to conventional pain relief. Not used in cases of combined gastrointestinal manifestations.
Dosage: Prednisolone 1 - 2mg / kg body weight / day for 3-4 weeks, then use every other day or gradually reduce the dose. Can be used alone or in combination with other immunosuppressants. Recently, some authors recommend the use of short-term high-dose corticosteroids to treat this condition (pulse therapy), with cases of severe kidney damage such as glomerulonephritis with nephrotic syndrome bolus corticoid may be used (both block) dose of 500-1,000mg methyl prednisolone / 24h for 3 consecutive days then reduce to the usual dose 2mg / kg / 24h and reduce the dose gradually, this method works well to improve clinical symptoms as well. as microscopic, reducing total corticosteroid dose as well as length of hospital stay.
Used in cases of severe kidney damage such as glomerulonephritis with extravascular proliferation affecting> 75% of glomeruli. The commonly used preparations are azathioprine 3 - 4 mg / kg / 24h in combination with corticosteroids to reduce dose over 6 months to 1 year, or cyclophosphamide can be used.
Penicillin is sometimes effective when it is caused by streptococcal infection.
Applies to end-stage failure of the body but with recurrence of allergic capillary nephritis in some patients who have had a kidney transplant.
Other measures have been used, although the effect is not clear
Plasma filtration, antihistamines are used but not proven effectiveness, an antispasmodic additive in cases of damage to the digestive system.