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The kidneys are affected in up to 80% of cases with signs of blood and protein in the urine and the injury can lead to either rapidly or slowly progressive kidney failure. The lungs are affected in 20-50% of cases with findings of pulmonary hemorrhage, or chronic pulmonary fibrosis leading to respiratory failure. [3]
If left untreated, it rapidly progresses into acute kidney failure [6] and death within months. In 50% of cases, RPGN is associated with an underlying disease such as Goodpasture syndrome, systemic lupus erythematosus or granulomatosis with polyangiitis; the remaining cases are idiopathic.
Long-term complications are common (86%), mainly chronic kidney failure, hearing loss, and deafness. [12] The risk of relapse is increased in people with GPA who test positive for anti-PR3 ANCA antibodies and is higher than the relapse risk for microscopic polyangiitis. [7]
This damage leads to inflammation in the glomerulus and can present with a nephritic syndrome. [27] Other ANCA small-vessel vasculitides - The conditions included in this category are eosinophilic granulomatosis with polyangiitis, microscopic polyangiitis, and granulomatosis with polyangiitis. [9]
The negative immunofluorescence pattern, however, is called "pauci-immune" and is often associated with systemic vasculitides (plural of vasculitis) including: microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis (EGPA), and granulomatosis with polyangiitis (GPA). [1] [2]
Type II is today more commonly known as dense deposit disease (DDD). [5] Most cases of dense deposit disease do not show a membranoproliferative pattern. [ 6 ] It forms a continuum with C3 glomerulonephritis ; together they make up the two major subgroups of C3 glomerulopathy .
Eosinophilic granulomatosis with polyangiitis consists of three stages, but not all patients develop all three stages or progress from one stage to the next in the same order; [7] whereas some patients may develop severe or life-threatening complications such as gastrointestinal involvement and heart disease, some patients are only mildly affected, e.g. with skin lesions and nasal polyps. [8]
Microscopic haematuria with or without proteinuria may be seen in Class II Lupus nephritis. Hypertension, nephrotic syndrome, and acute kidney injury are very rare at this stage. [6] Idiopathic mesangial proliferative glomerulonephritis is less established in the literature.