Schönlein-Henoch disease: symptoms

Hemorrhagic vasculitis (Chenlen-Henoch purpura disease) in most cases is a benign disease prone to spontaneous remissions or cure within a few weeks from the time of onset. However, in some patients, mainly adults, the disease acquires a recurring course with the development of severe kidney damage.

Characteristic extrarenal symptoms of Shenlaine-Henoch disease (skin, joint, GI tract) can appear in any sequence for several days, weeks or at the same time.

• Skin lesion is the main diagnostic symptom of purpura Schönlein-Henoch, which in half of patients is the first sign of the disease. There is a hemorrhagic rash (palpable purpura) on the extensor surfaces of the lower extremities, buttocks, less often on the abdomen, back and arms. The rashes are located symmetrically, initially on the ankles and heels, then begin to spread proximally. In severe cases, the rashes merge, acquire a generalized character. In children, the appearance of purpura is accompanied by swelling, even in the absence of proteinuria, which is believed to be associated with increased vascular permeability due to vasculitis. A rare form of rashes is a blister with hemorrhagic contents and subsequent desquamation of the epidermis. During the first weeks of the disease, purpura episodes recur, and at the time of relapse, as a rule, articular syndrome or gastrointestinal lesions appear for the first time. In a small part of the patients, purpura acquires a chronic recurrent course.
• Defeat joints in two-thirds of patients with purple Shonlein-Genoch. Most patients have symptoms such as Shonlein-Henoch purpura, such as migrating arthralgia of the knee and ankle joints, less often the joints of the upper limbs, combined with myalgias. Arthritis develops extremely rarely. In a small part of the patients, the articular syndrome is formed earlier than purpura.
• Lesions of the gastrointestinal tract are observed in more than 65% of patients with hemorrhagic vasculitis, and in some patients it is the first sign of the disease. Clinically, the gastrointestinal vasculitis manifests itself as symptoms of dyspepsia, intestinal bleeding, and abdominal pain. Severe complications develop in no more than 5% of cases; especially in older children, intussusception is noted. Small bowel infarctions with perforations, acute appendicitis, pancreatitis are also described. The late complication of the lesion of the gastrointestinal tract with purple Shoinlein-Henoch is the stricture of the small intestine.
• The rare extrarenal symptoms of purpura are the defeat of the lungs (decreased functional parameters, pulmonary hemorrhage, hemothorax), central nervous system (headaches, encephalopathy, convulsive syndrome, hemorrhagic stroke, behavioral disorders), urological pathology (ureteral stenosis, edema and scrotal hemorrhage, hematoma of the spermatic cord, necrosis of the testicles, hemorrhagic cystitis).

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Purple Shenolein-Genocha: renal damage

Kidney damage is a frequent, but not a permanent symptom of purple Shonlein-Genoch. Glomerulonephritis develops on average in 25-30% of patients. In adult patients with hemorrhagic vasculitis, its frequency reaches 63%.

Usually, the symptoms of kidney damage with Schönlein-Henoch purpura are found already in the onset of the disease, combining with the first episode of purpura or appearing soon after it. However, in some patients, glomerulonephritis may precede purpura, or develop for the first time a few years after the manifestation of vasculitis, without being associated with skin or joint syndromes. The severity of extrarenal symptoms does not correlate clearly with the severity of the renal process. Sometimes severe glomerulonephritis can develop with a moderate lesion of the skin and GI tract.

The most frequent clinical symptom of glomerulonephritis is erythrocyturia, which can be represented by both recurrent macrohematuria and persistent microhematuria. Macrogematuria may accompany recurrence of purpura or be isolated in isolation, usually in the presence of nasopharyngeal infection. In most patients, hematuria is combined with proteinuria, which is usually small in the general population of patients with hemorrhagic vasculitis. However, in patients with IgA-nephritis with Schönlein-Henoch purpura, who are in nephrologic departments due to the severity of renal damage, massive proteinuria with formation of a nephrotic syndrome is most often noted in 60% of cases. Early impairment of kidney function and hypertension are usually detected in patients with nephrotic syndrome. Renal insufficiency is moderately expressed. In adult patients with purple Shoinlein-Genoch, unlike children, more severe renal damage occurs in the form of a rapidly progressive glomerulonephritis. In these cases, kidney biopsy reveals diffuse proliferative or extracapillary glomerulonephritis with half-moon in a large percentage of the glomeruli, while the bulk of patients with hematuria and moderate proteinuria have a morphological picture of mesangioproliferative glomerulonephritis.