Schoenlein-Genoch disease - Symptoms.

Hemorrhagic vasculitis (Schonlein-Henoch purpura disease) is in most cases a benign disease, prone to spontaneous remissions or recovery within a few weeks from the moment of occurrence. However, in some patients, mainly adults, the disease acquires a recurrent course with the development of severe kidney damage.

Characteristic extrarenal symptoms of Henoch-Schonlein disease (skin, joint, and gastrointestinal lesions) may appear in any sequence over several days, weeks, or simultaneously.

• Skin lesions are the main diagnostic symptom of Henoch-Schonlein purpura, which is the first sign of the disease in half of the patients. Hemorrhagic rash (palpable purpura) appears on the extensor surfaces of the lower extremities, buttocks, and less often on the abdomen, back, and arms. The rash is symmetrical, initially on the ankles and heels, then begins to spread proximally. In severe cases, the rash merges and becomes generalized. In children, the appearance of purpura is accompanied by edema even in the absence of proteinuria, which is believed to be associated with increased vascular permeability due to vasculitis. A rare form of rash is vesicles with hemorrhagic contents and subsequent desquamation of the epidermis. During the first weeks of the disease, episodes of purpura recur, and at the time of relapse, as a rule, joint syndrome or gastrointestinal tract damage occurs for the first time. In a small number of patients, purpura becomes chronically relapsing.
• Joint damage is observed in two thirds of patients with Henoch-Schonlein purpura. Most patients experience symptoms of Henoch-Schonlein purpura such as migrating arthralgia of the knee and ankle joints, less often of the joints of the upper extremities, combined with myalgia. Arthritis develops extremely rarely. In a small proportion of patients, articular syndrome develops before purpura.
• Gastrointestinal tract lesions are observed in more than 65% of patients with hemorrhagic vasculitis, and in some patients it is the first sign of the disease. Clinically, gastrointestinal vasculitis is manifested by symptoms of dyspepsia, intestinal bleeding, and abdominal pain. Severe complications develop in no more than 5% of cases; intussusception intestinal obstruction is especially common in older children. Small intestinal infarctions with perforation, acute appendicitis, and pancreatitis have also been described. A late complication of gastrointestinal tract lesions in Henoch-Schonlein purpura is small intestinal stricture.
• Rare extrarenal symptoms of Henoch-Schonlein purpura include lung damage (decreased functional parameters, pulmonary hemorrhage, hemothorax), CNS (headaches, encephalopathy, convulsive syndrome, hemorrhagic stroke, behavioral disorders), urological pathology (ureteral stenosis, swelling and hemorrhage in the scrotum, spermatic cord hematoma, testicular necrosis, hemorrhagic cystitis).

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Henoch-Schonlein Purpura: Kidney Damage

Kidney damage is a common but not constant symptom of Henoch-Schonlein purpura. Glomerulonephritis develops in an average of 25-30% of patients. In adult patients with hemorrhagic vasculitis, its frequency reaches 63%.

Usually, symptoms of kidney damage in Henoch-Schonlein purpura are detected already at the onset of the disease, combined 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 several years after the manifestation of vasculitis, regardless of skin or joint syndromes. The severity of extrarenal symptoms does not clearly correlate with the severity of the renal process. Sometimes severe glomerulonephritis can develop with moderate damage to the skin and gastrointestinal tract.

The most common clinical symptom of glomerulonephritis is erythrocyturia, which may be presented as both recurrent macrohematuria and persistent microhematuria. Macrohematuria may accompany relapses of purpura or be observed in isolation, usually in the presence of a 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 in Henoch-Schonlein purpura, who are in nephrology departments due to the severity of renal damage, massive proteinuria with the formation of nephrotic syndrome in 60% of cases is most often noted. Early renal dysfunction and arterial hypertension are usually detected in patients with nephrotic syndrome. Renal failure is moderate. In adults with Henoch-Schonlein purpura, as opposed to children, severe kidney damage is more often observed, occurring in the form of rapidly progressive glomerulonephritis. In these cases, renal biopsy reveals diffuse proliferative or extracapillary glomerulonephritis with crescents in a large percentage of glomeruli, while the majority of patients with hematuria and moderate proteinuria have a morphological picture of mesangioproliferative glomerulonephritis.