Pulmonary hemorrhage with hematuria: do not forget IgA nephropathy

Oluwole, Kemi; Esuzor, Linda; Adebiyi, Oluwafisayo; Nzerue, Chike; Faulkner, Marquetta; Umeukeje, Ebele M.; Paueksakon, Paisit

doi:10.1093/ckj/sfs095

Cited by 5 publications

(6 citation statements)

References 21 publications

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“…[28][29][30][31][32][33][34][35][36][37][38][39][40][41][42][43][44][45][46][47] We excluded one report as no renal biopsy was done to establish the diagnosis of IgAN. 37 Our final review had 23 patients in 19 case reports; this included 19 reports of DAH, 28,29,[33][34][35][38][39][40][41][42][43][44][45][46][47] two reports of interstitial lung disease 31,32 and organizing pneumonia 30,36 each (Table S1). Two patients had findings consistent with organizing pneumonia on SLBx, although this was reported as bronchiolitis obliterans or immune complex pneumonitis; 30,36 both these patients had demonstrable IgA on surgical lung biopsy, indicating causality of this association.…”

Section: Our Data and Results Of Systematic Searchmentioning

confidence: 99%

“…Dyspnea (84%), hemoptysis (74%), cough (53%) and fever (47%) were the most common presenting complaints; 26.3% (5/19) had prominent symptoms suggestive of a connective tissue disease that was excluded on subsequent evaluation 33,34 or had an inactive connective tissue disease. 38,44 Simultaneous involvement of both kidneys and lungs was the most common presenting manifestation (42%); 29,34,35,38,40,41,45 one-third (37%) had prior diagnosed kidney disease 39,42,46,47 or undiagnosed IgAN 33 before presenting with PRS. In 21%, presentation was with alveolar hemorrhage.…”

Section: Our Data and Results Of Systematic Searchmentioning

confidence: 99%

“…Steroids with cyclophosphamide, followed by maintenance with methotrexate or azathioprine was used in 44.4% (8/19). 34,[38][39][40][42][43][44][45] No therapy was administered in two patients because of lack of recognition of DAH 33 or physician's perception of lack of benefit. 34 Rituximab was used in one patient, without reported clinical benefit.…”

Section: Our Data and Results Of Systematic Searchmentioning

confidence: 99%

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Diffuse alveolar hemorrhage in IgA nephropathy: case series and systematic review of the literature

et al. 2016

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These findings are similar to our previous observations of Henoch-Schonlein purpura (HSP)-related alveolar hemorrhage, highlighting the similarities of these related syndromes. Multicentric studies of IgAN and HSP-related pulmonary renal syndrome with a standard protocol are needed to define their similarities and differences, optimum suppression and its role in preventing renal progression in this setting.

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Section: Our Data and Results Of Systematic Searchmentioning

confidence: 99%

Section: Our Data and Results Of Systematic Searchmentioning

confidence: 99%

Section: Our Data and Results Of Systematic Searchmentioning

confidence: 99%

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Diffuse alveolar hemorrhage in IgA nephropathy: case series and systematic review of the literature

et al. 2016

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“…Ig A nephropathy rarely presents as concomitant renal and pulmonary findings. The most common reported form of pulmonary involvement is pulmonary hemorrhage with probable alveolar capillaritis (3,6). As far as we know there is no patient with IgA nephropathy in literature that has pulmonary cavitary lesions as one of the presenting features.…”

Section: N Discussionmentioning

confidence: 99%

Pulmonary cavitary lesions may be one of the presenting features in Ig A nephropathy

Isci

Gamsiz²,

Kayıpmaz³

et al. 2018

Reumatismo

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Immunoglobulin A (Ig A) nephropathy is the most frequent primary glomerulonephritis. Renal limited disease is the most widespread clinical form of the disease. Pulmonary involvement may also be seen concomitantly and capillaritis with pulmonary hemorrhage is the most frequent pulmonary involvement. In this paper, for the first time in literature, we describe an Ig A nephropathy patient with multiple pulmonary cavities as one of the presenting features of the disease. Also, no other etiology for the cavities was found other than Ig A nephropathy. Herein, possible pathogenesis might be capillaritis or deposition of immune complexes. As a result, it should be kept in mind that pulmonary cavity may be the presenting feature of Ig A nephropathy especially with other frequent signs of the disease.

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“…Since the respiratory tract is also lined with mucosa, the predominant immunoglobulin protecting the respiratory tract is IgA. As such, infections involving the lung causing vasculitides, bronchiolitis, capillaritis, alveolitis, and pulmonary hemorrhage (diffuse alveolar hemorrhage), and even tuberculosis, though less common, have caused secondary IgA nephropathy [ 36 ]. In some cases, pulmonary diseases seem to resolve when they are treated with corticosteroids for the IgA nephropathy, suggesting the existence of a pulmonary-kidney axis [ 37 ].…”

Section: Reviewmentioning

confidence: 99%

Progress in Pathogenesis of Immunoglobin A Nephropathy

Stanley¹,

Deng²

2020

Cureus

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The understanding of the pathogenesis of any disease is the key to effective and specific treatment of the disease. immunoglobulin A (IgA) nephropathy is an autoimmune disease of the kidney. Oxford MEST classification is commonly used to stratify patients according to the severity of the disease. Patients with IgA nephropathy seem to produce anti-GalNAc antibodies against a particularly defective IgA1. This immune complex deposits in the kidneys, leading to a type 3 hypersensitivity reaction which ultimately damages the kidneys. People of a certain genetic background and who experience upregulation of certain defective receptors seem to develop primary IgA nephropathy. Secondary IgA nephropathy could be due to dysbiosis of the microbiota in the gut, compromised gut immunity or other gut pathologies, pulmonary function abnormalities, or amyloidosis. Overproduction of IgA due to plasma cell dyscrasia or reduced clearance of IgA due to liver abnormalities could also be potential causes. Genes that predispose individuals to IgA nephropathy and intestinal abnormalities, such as Celiac disease, seem to overlap and these people tend to have a poorer prognosis and need to be placed on more intensive treatment regimens. IgA Vasculitis seems to be a systemic form of IgA nephropathy, whereby IgA deposits systemically and leads to multiple disease manifestations. Patients in high-risk groups could also be prophylactically screened for the disease and closely monitored by immunohistochemical methods such as an enzyme-linked immunosorbent assay (ELISA) or identified by genetic testing. Currently, the major treatment regimens involve supportive therapy or immunosuppressive therapy which has major side effects. More specific treatment methods such as monoclonal antibodies, immunoglobulin replacement therapy, or low-antigen-content diet could also be looked into as potential treatment options. Stem cell replacement, by way of bone marrow transplant and tonsillectomy, has been suggested as a treatment option in patients with indications.

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Pulmonary hemorrhage with hematuria: do not forget IgA nephropathy

Cited by 5 publications

References 21 publications

Diffuse alveolar hemorrhage in IgA nephropathy: case series and systematic review of the literature

Diffuse alveolar hemorrhage in IgA nephropathy: case series and systematic review of the literature

Pulmonary cavitary lesions may be one of the presenting features in Ig A nephropathy

Progress in Pathogenesis of Immunoglobin A Nephropathy

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