Symptoms of kidney damage in Wegener's granulomatosis

The onset of Wegener's granulomatosis often occurs as a flu-like syndrome, the development of which is associated with the circulation of proinflammatory 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, migrating 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.

The symptoms of Wegener's granulomatosis, as with other small vessel vasculitis, are distinguished by significant polymorphism associated with frequent damage to the vessels of the skin, lungs, kidneys, intestines, and peripheral nerves. The frequency of these organ manifestations varies with different forms of small vessel vasculitis.

• Upper respiratory tract lesions are pathognomonic for Wegener's granulomatosis. The first symptoms of Wegener's granulomatosis are usually ulcerative-necrotic rhinitis with purulent discharge, but sinusitis and otitis media may develop. Over time, perforation of the nasal septum due to its necrosis and saddle-shaped deformation of the nose due to cartilage destruction are possible. Tracheal lesions (a rare symptom in adults) are clinically manifested by hoarseness and stridor breathing. In the most severe cases, laryngeal stenosis may develop. In children, these symptoms are noted in 50% of cases.
• Lung damage is the second pathognomonic sign of Wegener's granulomatosis. Clinical manifestations (cough, dyspnea, chest pain, hemoptysis) are observed in only half of the patients; in the rest, only radiographic changes are detected. 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 experience secondary infection with the development of pneumonia, abscess formation of cavities.
• Eye damage in the form of episcleritis, uveitis, iritis is observed in 50% of patients. The most serious disorder is granulomatosis of the orbit, leading to exophthalmos. Retrobulbar 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 dermal vessels. The most common symptom is palpable purpura on the skin of the lower extremities. In addition, ulcerated nodules, petechiae, and ecchymosis are noted.
• More than half of patients with Wegener's granulomatosis have muscle damage, manifested by pain. Increased levels of creatine phosphokinase, indicating necrosis, are extremely rare. Myalgia is based on muscle ischemia due to necrotizing inflammation of small vessels.
• Damage to the nervous system may be represented by pathology of the peripheral nerves and the central nervous system. Most often, multiple mononeuritis is detected as a result of vasculitis of small epineural vessels, leading to nerve ischemia. A small number of patients develop damage to the cranial nerves due to the spread of the process from the nasopharynx and middle ear to the base of the skull. Damage to the II, VI and VII cranial nerves is most typical. Damage to the meninges due to granulomatous inflammation is a rare symptom.
• Gastrointestinal tract damage manifests itself in pain and dyspeptic disorders, associated mainly with pathology of the small intestine. Ulcers in the intestine may develop, accompanied by bleeding.

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Symptoms of Wegener's granulomatosis: kidney damage

• Clinically, kidney damage in patients with Wegener's granulomatosis may manifest itself in various nephrological syndromes, ranging from asymptomatic proteinuria and/or hematuria to unprogressive glomerulonephritis depending on the morphological changes. Urinary syndrome in patients with Wegener's granulomatosis may be represented by persistent microhematuria and proteinuria.
  • Hematuria is a constant symptom of the disease. Red blood cell casts are often found in the urine sediment. Macrohematuria is rare.
  • Proteinuria is usually moderate, not exceeding 2-3 g/day. Massive proteinuria with the formation of nephrotic syndrome is often detected.
• In some patients, acute nephritic syndrome with moderate arterial hypertension and deterioration of renal function develops at the onset. Later, it transforms into nephrotic syndrome. In most cases of ANCA-associated vasculitis, kidney damage manifests as rapidly progressive glomerulonephritis with increasing renal failure. In a small number of patients, slow progression of renal failure is noted. Arterial hypertension develops in 50% of patients with Wegener's granulomatosis with kidney damage.
• In almost 20% of patients with ANCA-associated glomerulonephritis, hemodialysis is required already at the first admission to a nephrology hospital. The reasons for this may be either maximum activity of vasculitis, leading to a rapid deterioration in renal function due to severe morphological changes (necrosis, crescents in 100% of glomeruli), or terminal chronic renal failure, the accelerated development of which is associated with a delayed start of treatment. In the first case, active immunosuppressive therapy can lead to normalization of renal function and cessation of hemodialysis treatment in most patients.