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Pulmonary Renal Syndrome: Update Article International Journal of Health Sciences and Research www.ijhsr.org ISSN: 2249-9571 Review Article Pulmonary Renal Syndrome: Update Article N.S.Neki1, Satpal Aloona2 1Professor, 2Assistant Professor, Department of Medicine, Govt. Medical College/ Guru Nanak Dev Hospital, Amritsar, India- 143001 Corresponding Author: N.S. Neki Received: 08/01/2017 Revised: 24/01/2017 Accepted: 30/01/2017 ABSTRACT The pulmonary–renal syndrome (PRS) refers to the combination of diffuse alveolar haemorrhage and rapidly progressive glomerulonephritis (RPGN). Pulmonary-renal syndrome can originate from various systemic autoimmune diseases. ANCA-associated vasculitides account for approximately 60%, Goodpasture's Syndrome for approximately 20% of the cases. It is almost always autoimmune in nature, therefore steroids and other immunosupressants have role in its treatment. The underlying renal pathology is a form of focal proliferative glomerulonephritis. The lung pathology is in form of diffuse alveolar hemorrhages. Key Words: Pulmonary renal syndrome; Wegener's granulomatosis; microscopic polyangiitis; systemic lupus erythematosus. INTRODUCTION collagen. [3,4] The pulmonary-renal Pulmonary–renal syndrome (PRS) is syndrome (PRS) is a rare and life- defined as the combination of diffuse threatening condition. The clinical picture of alveolar haemorrhage (DAH) and PRS includes hemoptysis (not always glomerulonephritis. [1,2] PRS are caused by a present) due to alveolar hemorrhages, acute- wide variety of diseases, including various onset anemia and renal abnormalities forms of primary systemic vasculitis ranging from isolated urinary abnormalities (Wegener‟sgranulomatosis and microscopic to rapidly progressive glomerulonephritis. A polyangiitis), Goodpasture's syndrome significant number of patients will present which is associated with autoantibodies to with rapid clinical deterioration and require the alveolar and glomerular basement admission to the intensive care unit (ICU) membrane and systemic lupus This is attributed either to exacerbation of erythematosus. The diagnosis is mainly the disease activity itself, or to infectious based on the identification of particular complications secondary to severe patterns of clinical, pathologic, radiologic immunosuppressive treatment Diffuse and laboratory features. The majority of alveolar haemorrhage is characterized by cases of pulmonary-renal syndrome are the presence of a haemorrhagic associated with ANCAs, either c-ANCA or bronchoalveolar lavage (BAL) in serial p-ANCA, due to autoantibodies against the BAL samples. In the clinical setting of an target antigens proteinase-3 and acute nephritis syndrome, percutaneous myeloperoxidase respectively. The antigen renal biopsy is commonly performed for target in Goodpasture's syndrome is type IV histopathology and immunofluorescence International Journal of Health Sciences & Research (www.ijhsr.org) 294 Vol.7; Issue: 2; February 2017 N.S. Neki et. al. Pulmonary renal syndrome: update article studies. Treatment of generalized ANCA- A) Pulmonary–renal syndrome associated vasculitis consists of associated with anti-GBM corticosteroids and immunosuppressive antibodies: Goodpasture‟s syndrome agents. [5-7] (GPS) Pathology B) Pulmonary–renal syndrome in The underlying pulmonary lesion in the ANCA-positive systemic vasculitis majority of cases of pulmonary–renal 1. Wegener‟s granulomatosis(WG) syndrome is small-vessel vasculitis which is 2. Microscopic polyangiitis characterized by a destructive and 3. Churg–Strauss syndrome(CSS) inflammatory process that involves 4. Other vasculitis arterioles, venules and alveolar capillaries C) Pulmonary–renal syndrome in which. [8] These vasculitis have a ANCA-negative systemic vasculitis heterogeneous pathogenesis and there are 1. Henoch–Schönlein purpura three different pathophysiological 2. Mixed cryoglobulinaemia mechanisms of injury. [9] 3. Behçet‟s disease 1. mediated by anti-neutrophil-cytoplasmic 4. IgA nephropathy antibodies (ANCA), D) ANCA-positive pulmonary–renal 2. immune-complex mediated vasculitis of syndrome without systemic small vessels or vasculitis: Idiopathic pulmonary– 3. by antibodies against the glomerular renal syndrome basement membrane (Goodpasture Pauci-immune necrotizing syndrome). glomerulonephritis and pulmonary In the kidney, a rapidly progressive capillaritis glomerulonephritis (RPGN) is caused by E) Pulmonary–renal syndrome in drug- damage of the capillaries and basal associated ANCA-positive vasculitis membranes with leakage of erythrocytes, 1. Propylthiouracil which is followed by an influx of 2. D-Penicillamine macrophages, fibrinogen and the formation 3. Hydralazine of crescents.In the lungs, a diffuse alveolar 4. Allopurinol hemorrhage is caused by a pulmonary 5. Sulfasalazine capillaritis. [10] F) Pulmonary–renal syndrome in anti- In the case of ANCA-associated systemic GBM-postive and ANCA-positive vasculitis the ANCA is detected in about patients 80% of patients. Besides the correlation of G) Pulmonary–renal syndrome in ANCA titers with disease activity there is autoimmune rheumatic diseases evidence of a pathogenetic role of ANCA. (immune complexes and/or ANCA The ANCA is formed against two proteis mediated) named myeloperoxidase (MPO) and 1. Systemic lupus erythematosus proteinase 3 (Pr3) and these are detected in 2. Scleroderma (ANCA?) the cytoplasm of non-stimulated 3. Polymyositis neutrophils. Finally cell necrosis and 4. Rheumatoid arthritis apoptosis contribute to vascular 5. Mixed collagen vascular disease inflammation process.In Pauci-immune H) Pulmonary–renal syndrome in glomerulonephritis there is absence of thrombotic microangiopathy immune-complex deposition, 1. Antiphospholipid syndrome immunoglobulins or complement within the 2. Thrombotic thrombocytopenic biopsy sample. [11] purpura Classification of PRS according to the 3. Infections pathogenetic mechanism involved 4. Neoplasms (Etiopathological classification) [12] International Journal of Health Sciences & Research (www.ijhsr.org) 295 Vol.7; Issue: 2; February 2017 N.S. Neki et. al. Pulmonary renal syndrome: update article I) Diffuse alveolar haemorrhage (RPGN) and it is associated with anti-GBM complicating idiopathic pauci- antibodies. It is rare, however this syndrome immune glomerulonephritis is responsible for about 20% of acute renal failure due to consequences of RPGN. The Epidemiologic Aspects [13-16] disease is hereditary in nature as it has been Goodpasture‟s syndrome is not common described in brothers and in identical twins. and has an incidence of approximately 0.5 More than 80% of patients carry the HLA to 1 case per million people per year. alleles DR15 or DR4 whereas the alleles Gender distribution is equal in both sexes DR7 and DR1 are rarely found. and there is no gender predisposition. Environmental factors, such as smoking, Goodpasture‟s syndrome has a bimodal age infections and previous hydrocarbon distribution, with a large number of patients exposure, have been implicated in triggering presenting at ages 20 to 30 and at ages 50 to the disease. In GPS, the target antigen is the 68. Whites are more frequently affected non-collagenous (NC1) domain of the α3 than blacks. Antineutrophil cytoplasmic (IV) collagen chain, it is of one of the six autoantibody–associated vasculitis (ANCA) chains (alpha-1 to alpha-6 [α1 to α6]) which is the most common primary systemic small are entitled in making type IV collagen. vessel vasculitis to occur in adults. This target antigen is primarily found on the Although the etiology is sometimes inert aspect of the lamina densa, which is unknown, the incidence of vasculitis is the middle layer of the glomerular and increasing, and the diagnosis and alveolar basement membranes. Anti-GBM management of patients is challenging antibodies bind the glomerular basement because it is relatively infrequent, and has membrane, activating compliment and variable clinical expression. In patients proteases, resulting in the disruption of the presenting with PRS secondary to systemic filtration barrier and Bowman‟s capsule and vasculitis, pulmonary hemorrhage appears causing proteinuria and crescent formation. in 40% of WG cases and 30% of (ii)Pulmonary–renal syndrome in ANCA- microscopic polyangitis (MPA) cases, and it positive systemic vasculitis [23-27] rises when renal involvement is severe. Circulating ANCA autoantibodies are Pulmonary hemorrhage in these disorders detected in the majority of patients carries a mortality rate of 10%. Diffuse presenting with PRS. ANCA is not alveolar hemorrhage in systemic lupus confirmatory for specific type but it leads to erythematosus (SLE) remains a devastating differential diagnosis to three major pulmonary complication; mortality rates are systemic vasculitides syndromes which are around 45- 50%, and it occurs in less than associated with ANCA, includes: 2% of patients with SLE. Evidence for Wegener‟s granulomatosis, microscopic glomerular involvement is also present in polyangiitis and Churg–Strauss syndrome. large number of patients. Single-center The suspicion of a pulmonary-renal experience suggests that 60% to 70% of syndrome in an ANCA-associated systemic cases with PRS are associated with ANCAs, vasculitis can often be taken from a careful and 20% are associated with anti-GBM history and thorough clinical examination antibodies. [17,18] with detection of other vasculitic signs like Pathophysiology of PRS with respect to eye inflammation, intractable rhinitis / each pathological type sinusitis, skin rashes, arthralgia, myalgia
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