Anti-hLAMP2-antibodies and dual positivity for anti-GBM and MPO-ANCA in a patient with relapsing pulmonary-renal syndrome

Etter, Christoph; Gaspert, Ariana; Regenass, Stephan; Wüthrich, Rudolf P.; Kistler, Thomas; Kain, Renate; Cohen, Clemens D.

doi:10.1186/1471-2369-12-26

Cited by 9 publications

(6 citation statements)

References 16 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Dual positive patients have higher relapse rate then those with anti-GBM antibodies alone [6,9]. This suggests that an early indicator of disease relapse is needed in monitoring disease activity in double-positive patients, such as anti-hLAMP2 antibody [36,37]. …”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Pulmonary renal syndrome in a child with coexistence of anti-neutrophil cytoplasmic antibodies and anti-glomerular basement membrane disease: case report and literature review

et al. 2013

View full text Add to dashboard Cite

BackgroundPulmonary renal syndrome (PRS), denoting the presence of diffuse alveolar hemorrhage and glomerulonephritis as manifestations of systemic autoimmune disease, is very rare in childhood. The coexistence of circulating anti-neutrophil cytoplasmic antibody (ANCA) and anti-glomerular basement membrane (GBM) disease in children affected by this syndrome is exceptional, with unfavorable outcome in five out of seven patients reported to date. We describe a child with PRS associated with both circulating anti-myeloperoxidase (anti-MPO) ANCA and anti-GBM disease on renal biopsy who was successfully treated with immunosuppressive therapy.Case presentationA 10-year old girl presented with fever, fatigue, malaise, and pallor followed by hemoptysis and severe anemia. Diffuse alveolar hemorrhage was revealed on fiberoptic bronchoscopy. Renal findings consisted of microscopic hematuria, moderate proteinuria, and anti-GBM disease on renal biopsy. ANCA with anti-MPO specificity were present whereas anti-GBM antibodies were on borderline for positivity. Methyl-prednisolone pulses followed by prednisone led to cessation of hemoptysis, marked improvement of lung fuction, and normal finding on chest x-ray within 10 days. An immunosuppressive regimen was then given consisting of prednisone daily for 4 weeks with subsequent taper on alternate day, i.v. cyclophosphamide pulses monthly for 6 doses, followed by mycophenolate mofetil that resulted in normal lung function tests, hemoglobin concentration, and anti-MPO level within four subsequent weeks. During 10-months of follow-up she remained well, her blood pressure and renal function tests were normal, and proteinuria and hematuria gradually resolved.ConclusionWe report a child with an exceptionally rare coexistence of circulating ANCA and anti-GBM disease manifesting as PRS in whom renal disease was not the prominent part of clinical presentation, contrary to other reported pediatric patients. A review of literature on disease with double positive antibodies is also presented. Evaluation of a patient with PRS should include testing for presence of different antibodies. An early diagnosis and rapid institution of aggressive immunosuppressive therapy can induce remission and preserve renal function. Renal prognosis depends on the extent of kidney injury at diagnosis and appropriate treatment.

show abstract

Section: Discussionmentioning

confidence: 99%

“…The recurrence of disease activity with elevation of MPO-ANCA can occur many months after clinical remission, affecting either both lungs and kidneys or only one of these organs [6,12,13,31,32,41]. Most relapses responded well to methylprednisolone and/or cyclophosphamide therapy [12,36]. …”

Section: Discussionmentioning

confidence: 99%

Pulmonary renal syndrome in a child with coexistence of anti-neutrophil cytoplasmic antibodies and anti-glomerular basement membrane disease: case report and literature review

et al. 2013

View full text Add to dashboard Cite

show abstract

“…There are at least two reported cases which have dual positivity for anti-GBM antibody and ANCA with MN [14,15]. One case [14] had both perinuclear and cytoplasmic ANCAs, and the other [15] had antibodies to both human lysosomal membrane protein 2 (anti-hLAMP-2) and MPO-ANCA.…”

Section: Discussionmentioning

confidence: 99%

“…One case [14] had both perinuclear and cytoplasmic ANCAs, and the other [15] had antibodies to both human lysosomal membrane protein 2 (anti-hLAMP-2) and MPO-ANCA. The significance of the dual positivity of perinuclear and cytoplasmic ANCA or MPO-ANCA and anti-hLAMP-2 antibody is unclear.…”

Section: Discussionmentioning

confidence: 99%

Combined membranous nephropathy and crescentic glomerulonephritis with concurrent anti-glomerular basement membrane antibody and myeloperoxidase-specific anti-neutrophil cytoplasmic antibody

Iwafuchi¹,

Morita

Oyama³

et al. 2013

CEN Case Rep

View full text Add to dashboard Cite

We report a case of a 71-year-old man with rapidly progressive nephritic syndrome and dual positivity for anti-glomerular basement membrane antibody and myeloperoxidase-specific anti-neutrophil cytoplasmic antibody. Renal biopsy revealed crescentic, mainly cellular, glomerulonephritis with granulomatous lesions, and advanced membranous changes. Membranous nephropathy had apparently existed for an extended period before the development of crescentic glomerulonephritis. In some studies reporting the simultaneous occurrence of both diseases, membranous nephropathy might be followed by crescentic glomerulonephritis, presumably from a histological point of view. Although we cannot prove a causal relationship between the two diseases, we caution that precise observations, especially histological, are necessary in similar cases.

show abstract

“…Anti-EEA1 antibodies are seen in patients with neurological diseases and quantitated using an addressable laser bead immunoassay on Luminex platform [ 15 ]; anti-LAMP2 antibodies have been also recently studied in ANCA associated vasculitis [ 16 ]. Overall, the increasing interest for these “minor” autoantibodies may lead to the development of easier and more practicable methods for their detection.…”

Section: Discussionmentioning

confidence: 99%

Characterization for anti-cytoplasmic antibodies specificity by morphological and molecular techniques

Alpini

Lotzniker

Valaperta

et al. 2012

Autoimmun Highlights

View full text Add to dashboard Cite

PurposeThe aim of our study was the characterization of anti-cytoplasmic antibodies by home-made morphological and biochemical techniques. Indeed, indirect immunofluorescence (IIF) on HEp-2 cell line is not always exhaustive in relation to the complexity of the antigens involved.MethodsNine serum samples with anti-cytoplasmic antibodies (2 anti-Golgi apparatus, 3 with diffuse pattern and 4 with lysosome/endosome-like pattern) were tested with fluorescent confocal microscopy, Western blot analysis and, when necessary, with electron microscopy technique.ResultsConfirmation of the IIF staining pattern was performed in confocal microscopy by comparison with the respective antibody marker. The anti-endoplasmatic reticulum positivity was also confirmed by electron microscopy evaluation. Both anti-lysosome/endosome and anti-endoplasmatic reticulum positivity have been definitely identified by Western blot through clear reactivity with calreticulin and LC3B, respectively.ConclusionsThese results do not aim at representing a standard routine laboratory procedure. Electron microscopy evaluation cannot be proposed as a routine approach, but confocal microscopy technique may be offered in centralized reference laboratories. Newer technologies, especially multiplex immunoassay, can also lead to an easier identification of these autoantibodies, without recurring to a home-made immunoblotting. Only with a complete characterization we will be able to define the clinical relevance of anti-cytoplasmic antibodies, which are still considered as “esoteric” and not as “diagnostic” antibodies.

show abstract

Anti-hLAMP2-antibodies and dual positivity for anti-GBM and MPO-ANCA in a patient with relapsing pulmonary-renal syndrome

Cited by 9 publications

References 16 publications

Pulmonary renal syndrome in a child with coexistence of anti-neutrophil cytoplasmic antibodies and anti-glomerular basement membrane disease: case report and literature review

Pulmonary renal syndrome in a child with coexistence of anti-neutrophil cytoplasmic antibodies and anti-glomerular basement membrane disease: case report and literature review

Combined membranous nephropathy and crescentic glomerulonephritis with concurrent anti-glomerular basement membrane antibody and myeloperoxidase-specific anti-neutrophil cytoplasmic antibody

Characterization for anti-cytoplasmic antibodies specificity by morphological and molecular techniques

Contact Info

Product

Resources

About