Rapidly progressive glomerulonephritis
In the rapidly progressive glomerulonephritis ( engl. proceed rapidly progressive glomerulonephritis, RPGN short ) is a relapsing- occurring extracapillary glomerulonephritis, which is accompanied by a rapid loss of renal function. If left untreated, it can lead to renal failure within weeks to months. Other synonyms for this disease are rapidly progressive glomerulonephritis and crescentic glomerulonephritis (English crescentic glomerulonephritis ), and necrotizing intra-/extrakapilläre-proliferierende GN with crescent formation.
- 7.1 complications
- 8.1 Notes and references
- 8.2 Literature
- 8.3 External links
Epidemiology
Incidence: < 1/100.000 / year
Pathology
Pathogenesis
It is in the RPGN an immune complex glomerulonephritis or Antibasalmembran glomerulonephritis. The RPGN can occur for no apparent reason ( idiopathic) or more common in systemic diseases such as Wegener's granulomatosis, Goodpasture's syndrome, polyarteritis nodosa, or.
Macroscopic
The kidneys are slightly enlarged and may have some pinpoint bleeding on.
Microscopic
The histological picture that shows up is colorful, as the disease progresses in waves. Typical is the distinctive crescent formation in the glomeruli. Furthermore, individual monocytes are found. The area surrounding the glomerulus infiltrated most flammable. In the electron micrograph showing the Gomerulusschlingen ruptured and the cap space of the glomerulus is filled with fibrin. With a specific staining, the immunofluorescence let the plant pattern of immunoglobulins prove this is used to distinguish the various forms of RPGN (see below).
The ANCA -associated rapidly progressive glomerulonephritis is according to the percentage of glomeruli with crescents and inflammatory share the scarred glomeruli (global sclerosis) divided into:
This classification allows an estimation of prognosis: patients with focal ANCA-associated glomerulonephritis have the best prognosis. Patients with predominantly crescent formation have a high acute early stage of the disease, the chance that the disease responds to a immunusuppressive therapy, but are relatively good. The prognosis of mixed form is worse. Patients with a high proportion of scarred glomeruli are already at an advanced stage of the disease, the chance that this is responsive to treatment, are low.
Classification
There are three different forms of RPGN:
- Without lung involvement, very rare
- With lung involvement = Goodpasture's syndrome, often men before age 40
Clinic
- Rapid onset, hypertension,
- Renal failure to dialysis within weeks at ultrasound does not provide enlarged kidneys,
- Proteinuria with eventual nephrotic syndrome,
- Pulmonary hemorrhage in Goodpasture's syndrome
Diagnosis
The clinic and rapidly increasing retention values , immunological diagnosis ( see above). Renal biopsy with the following histology can confirm the diagnosis. The differential diagnoses include acute renal failure ( history of the triggering event ) and the acute nonbacterial nephritis, here then the medication should be obtained.
The exact determination antizytoplasmatischer antibodies against neutrophils (ANCA ) and corresponding distribution patterns can differentiate the cause of RPGN. In Wegener 's granulomatosis is c -ANCA find. The type p- ANCA should be rather thought of a microscopic polyangiitis.
Therapy
According to the immunopathogenesis is the therapy of high-dose immunosuppression with steroids in combination with cyclophosphamide. Goodpasture 's syndrome is treated in combination with plasma separation.
The renal failure occurring must be treated with dialysis.
Course and prognosis
The course and prognosis depends on the initiation of therapy. The presence of renal insufficiency requiring dialysis or a fibrosis of the interstitium are prognostically unfavorable.
In Goodpasture's syndrome (antibodies against the basement membranes of alveoli and glomeruli ) can together pulmonary and renal symptoms, but also occur separately. This leads to pulmonary infiltrates, hemoptysis, microcytic anemia and a progressive kidney failure.
Goodpasture 's syndrome, untreated, leads to death within a few days. With early diagnosis and treatment, the prognosis has improved considerably.
Complications
Development of terminal renal insufficiency with pulmonary edema, hypertension, hyperkalemia, possibly pulmonary hemorrhage.