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*For medical professionals to read and reference diseases affecting the whole body, be careful! Immunoglobulin A vasculitis (IgA vasculitis), formerly known as anaphylactoid purpura, is a small vasculitis characterized by the deposition of IgA-dominant immune complexes
.
More common in children, some adults are affected
.
The basic pathological changes are extensive small-vessel leukocytoclastic vasculitis, mainly capillary vasculitis, which can affect small veins and arterioles.
Commonly involved parts are skin, joints, gastrointestinal tract, and kidneys, and a few involve the heart, lungs and other organs.
IgA-dominated immune complex deposition was observed under immunofluorescence microscopy
.
The main clinical manifestations are skin, digestive tract, joint, and kidney involvement
.
01 Skin involvement is a common symptom and is a necessary condition for the diagnosis of IgA vasculitis
.
Typical purpura is similar to urticaria or red papules, distributed symmetrically on the limbs or buttocks, mainly on the extensor side, with obvious pressure points, especially on the ankles, which can gradually expand to the whole body, and can form herpes, necrosis and ulcers
.
Necrosis or hemorrhage occurs in about one-third of adults
.
After adequate rest, purpura can gradually disappear on its own after about 2 weeks, but can reappear as a chronic skin change
.
02 About two-thirds of patients with joint involvement may experience arthralgia, mainly involving the knee or ankle joint, but erosive arthritis is rare
.
Myalgia may also be associated, but serum creatine kinase is often within the normal range
.
03 Digestive tract involvement includes mild abdominal pain and/or vomiting, persistent abdominal cramping occurs in about one-third of patients, and serious complications include intussusception, infarction, and perforation
.
A recent Spanish study found that the main abdominal symptoms were abdominal pain (100%), nausea and vomiting (14.
4%), melena/rectal bleeding (12.
9%), and positive fecal occult blood (10.
3%)
.
Abdominal symptoms are often caused by intestinal ischemia and edema.
The duodenum and terminal ileum are often involved.
Endoscopically, diffuse mucosal congestion, petechiae, hemorrhagic erosions, and ulcers appear.
Abdominal CT shows intestinal wall thickening and mesentery.
Vascular congestion
.
04 Kidney involvement mostly occurs within one month after the onset of the rash, a few occur within a few months after the purpura subsides, and occasionally occurs before the skin purpura
.
Clinically, the incidence of renal involvement is about 45% to 85%.
Microscopic hematuria and/or proteinuria are common, and gross hematuria needs to exclude other diseases
.
About 1/3 of patients may develop hypertension, single or combined with acute glomerulonephritis or nephrotic syndrome, and renal failure may occur in severe cases
.
Kidney failure occurs in about 30% of adults with kidney involvement, and relatively rare in children
.
However, in all glomerulonephritis, IgA vasculitis nephropathy only accounts for 0.
6% to 2%
.
05 Involvement of other systems Rare manifestations of IgA vasculitis include orchitis, myocarditis, alveolar hemorrhage and scleritis, central and peripheral nervous system involvement may also occur, headache is more common, and convulsions, paralysis, chorea, ataxia, and aphasia may also occur , blindness, coma, subarachnoid hemorrhage, optic neuritis and Guillain-Barré syndrome, intracranial mass and hemorrhage have also been reported
.
IgA vasculitis usually presents as a benign self-limiting disease in children and is more severe in adults
.
Abdominal pain and fever are relatively uncommon in adults, but kidney involvement is more common and more severe
.
How is IgA vasculitis treated? Most of the disease has a good prognosis, and the treatment is mainly symptomatic
.
01 Symptomatic treatment of rash is the main treatment
.
02 When the only symptoms of joint pain are joint pain, non-steroidal anti-inflammatory analgesics can be added, and some patients with poor efficacy can be added with glucocorticoids
.
03 Symptomatic treatment is the main treatment for patients with mild gastrointestinal tract involvement, diet is controlled, and low-dose glucocorticoids should be added if necessary
.
For severe patients, it is necessary to actively evaluate the condition, treat with high-dose glucocorticoids and add immune preparations
.
04 Renal involvement should be treated in combination with clinical classification and pathological grading
.
Angiotensin-converting enzyme inhibitors are recommended for patients with mild to moderate proteinuria
.
Patients with massive proteinuria and acute renal failure advocate active use of glucocorticoids and immunosuppressive therapy
.
Source of this article: rheumatism leap month said responsible editor: cassette copyright statement This article is reproduced,