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Immunsuppressive treatment is the gold standard management in order to achieve remission of the blood vessel inflammation that occurs in active microscopic polyangitis. The current immunosuppressive protocols consists of a combination of high dose of glucocorticoids in combination with either cyclophosphamide or Rituximab . [ 8 ]
Type III RPGN may be isolated to the glomerulus (primary, or idiopathic) or associated with a systemic disease (secondary). In most cases of the latter, the systemic disease is an ANCA-associated vasculitis such as granulomatosis with polyangiitis, microscopic polyangiitis or eosinophilic granulomatosis with polyangiitis. [2]
Involvement of the ears, nose, and throat is more common in granulomatosis with polyangiitis than in the similar condition microscopic polyangiitis. [7] If the person has signs of kidney involvement or cutaneous vasculitis, a biopsy is obtained from the kidneys. Rarely, a thoracoscopic lung biopsy is required.
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]
Pulmonary-renal syndrome (PRS) is a rare medical syndrome in which respiratory failure involving bleeding in the lungs and kidney failure (glomerulonephritis) occur. [1] PRS is associated with a high rate of morbidity and death. [1] The term was first used by Goodpasture in 1919 to describe the association of respiratory and kidney failure. [1]
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 .
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.
As the glomerulonephritis progresses, the tubules of the kidney become infected, leading to atrophy and hyalinisation. The kidney appears to shrink. Treatment with corticosteroids is attempted if the disease progresses. [citation needed] In extremely rare cases, the disease has been known to run in families, usually passed down through the females.