Symptoms of kidney damage with Wegener's granulomatosis

The onset of the disease of Wegener's granulomatosis often proceeds in the form of an influenza-like syndrome, the development of which is associated with the circulation of pro-inflammatory cytokines, possibly produced as a result of a bacterial or viral infection preceding the prodromal period of the disease. At this time, most patients note typical symptoms of Wegener's granulomatosis: fever, weakness, malaise, migratory arthralgia in both large and small joints, myalgia, anorexia, weight loss. The prodromal period lasts about 3 weeks, after which the main clinical signs of the disease appear.

Symptoms of Wegener's granulomatosis, as in other vasculitis of small vessels, are distinguished by a significant polymorphism associated with frequent damage to the vessels of the skin, lungs, kidneys, intestines, peripheral nerves. With different forms of vasculitis of small vessels, the frequency of these organ manifestations is different.

• The defeat of the upper respiratory tract pathognomonic for Wegener's granulomatosis. The first symptoms of Wegener's granulomatosis, as a rule, are ulcerative necrotic rhinitis with a purulent discharge, but sinusitis, otitis media can develop. Over time, perforation of the nasal septum is possible due to its necrosis, saddle-shaped deformation of the nose due to destruction of the cartilage. Tracheal injury (a rare symptom in adults) is clinically manifested by the hoarseness of the voice, the stridor breathing. In the most severe cases, stenosis of the larynx can develop. In children, these symptoms are noted in 50% of cases.
• The defeat of the lungs is the second pathognomonic sign of Wegener's granulomatosis. Clinical manifestations (cough, shortness of breath, pain in the chest, hemoptysis) are noted only in half of the patients; the rest reveal only radiographic changes. Radiography reveals single or multiple rounded infiltrates. The disease is characterized by their migratory nature, rapid decay with the formation of cavities. Almost 50% of patients report secondary infection with the development of pneumonia, abscessing of the cavities.
• The defeat of the eyes in the form of episcleritis, uveitis, iritis is observed in 50% of patients. The most serious disorder is the granulomatosis of the orbit, leading to the appearance of exophthalmos. Retrobulbaric inflammation can lead to ischemia of the optic nerve and blindness.
• Skin lesions are observed in 40% of patients. It is based on leukocytoclastic angiitis of the vessels of the dermis. The most common symptom is palpable purpura on the skin of the lower limbs. In addition to this, nodules with ulceration, petechiae, and ecchymosis are noted.
• More than half of patients with Wegener's granulomatosis have muscular damage, manifested by pain. Increase in the level of creatine phosphokinase, indicating the necrosis, is noted very rarely. At the base of myalgias is ischemia of muscles due to necrotizing inflammation of small vessels.
• The defeat of the nervous system is represented by the pathology of the peripheral nerves and the central nervous system. The most common manifestation of multiple mononeurites as a result of vasculitis of small epineural vessels, leading to ischemia of the nerves. A small number of patients develop a lesion of the cranial nerves due to the spread of the process from the nasopharynx and the middle ear to the base of the skull. The most common lesion of II, VI and VII cranial nerves. The defeat of the meninges due to granulomatous inflammation is a rare symptom.
• The defeat of the gastrointestinal tract is manifested by pains and dyspeptic disorders, mainly associated with the pathology of the small intestine. Possible development of ulcers in the intestines, accompanied by bleeding.

[1], [2], [3], [4]

Symptoms of Wegener's granulomatosis: renal damage

• Clinically, renal damage in patients with Wegener's granulomatosis can be manifested by various nephrologic syndromes, ranging from asymptomatic proteinuria and / or hematuria to non-progressive glomerulonephritis, depending on morphological changes. The urinary syndrome in patients with Wegener's granulomatosis is represented by persistent microhematuria and proteinuria.
  • Hematuria is a constant sign of the disease. Often, erythrocyte cylinders are found in urine sediment. Macrogematuria is rarely noted.
  • Proteinuria, as a rule, is moderate, does not exceed 2-3 g / day. Often, massive proteinuria is detected with the formation of a nephrotic syndrome.
• In some patients, debility develops acute hypertension with moderate arterial hypertension and impaired renal function. Later, it is transformed into a nephrotic syndrome. In most cases of ANCA-associated vasculitis, kidney damage manifests a rapidly progressive glomerulonephritis with increasing renal failure. A small number of patients noted a slow progression of renal failure. Arterial hypertension develops in 50% of patients with Wegener's granulomatosis with renal involvement.
• Practically in 20% of patients with ANCA-associated glomerulonephritis, hemodialysis is necessary already at the first admission to the nephrological hospital. The reasons for this may be either the maximum activity of vasculitis, which led to a rapid deterioration in kidney function due to severe morphological changes (necrosis, half-moon in 100% of the glomeruli) or terminal chronic renal failure, the accelerated development of which is associated with delayed onset of treatment. In the first case, active immunosuppressive therapy can lead to normalization of kidney function and discontinuation of hemodialysis treatment in most patients.