scholarly journals A Case of Diffuse Alveolar Hemorrhage Associated with High-Titer of MPO-ANCA Demonstrating Cytoplasmic Staining Pattern

2019 ◽  
Vol 2019 ◽  
pp. 1-5
Author(s):  
Jihad Ben Gabr ◽  
Hiroshi Kato
Keyword(s):  
Staining Pattern ◽  
High Titer ◽  
Rapidly Progressive Glomerulonephritis ◽  
Diffuse Alveolar Hemorrhage ◽  
Alveolar Hemorrhage ◽  
Diagnostic Challenge ◽  
Presumptive Diagnosis ◽  
Cytoplasmic Staining ◽  
Life Threatening ◽  
Cytoplasmic Staining Pattern

Diffuse alveolar hemorrhage (DAH) is a life-threatening complication of ANCA-associated vasculitis (AAV) that requires urgent recognition and treatment. A presumptive diagnosis is often rendered without histopathology if concordant positivity of ANCA by indirect immunofluorescence (IIF) and ELISA assays, i.e., P-ANCA+/myeloperoxidase (MPO) Ab+ or C-ANCA+/proteinase-3 (PR3) Ab+, is documented in the context of pulmonary-renal syndrome or rapidly progressive glomerulonephritis. In this respect, the discordance between IIF and ELISA assays poses a diagnostic challenge in the absence of convincing histopathology and involves the risks of delaying the implementation of timely immunosuppressive therapy. Here, we report a 74-year-old woman who developed DAH and was found to have a high titer of MPO-ANCA exhibiting cytoplasmic staining on IIF, i.e., MPO-C-ANCA. The literature suggests that the availability of distinct epitopes on the MPO molecule dictates the perinuclear versus cytoplasmic staining pattern, which potentially explains the discordance between ELISA and IIF assays. Her DAH was controlled in association with seven sessions of plasmapheresis, methylprednisolone 1 gram daily for 3 days followed by 1 mg/kg/day, and rituximab. This case exemplifies the importance of consideration of pretest probability of suspected diagnosis that would realize a plausible interpretation of seemingly inconsistent serological profile and its effective incorporation into the diagnostic reasoning.

Antiglomerular Basement Membrane Disease

2018 ◽  
Vol 39 (04) ◽  
pp. 494-503 ◽  
Author(s):  
Charles Pusey ◽  
Stephen McAdoo
Keyword(s):  
Basement Membrane ◽  
Rapidly Progressive Glomerulonephritis ◽  
Basement Membranes ◽  
Alveolar Hemorrhage ◽  
Type Iv ◽  
Maintenance Immunosuppression ◽  
Life Threatening ◽  
Renal Prognosis ◽  
Function Testing

AbstractAntiglomerular basement membrane (anti-GBM) disease is a rare but life-threatening autoimmune vasculitis that is characterized by the development of pathogenic autoantibodies to type IV collagen antigens expressed in the glomerular and alveolar basement membranes. Once deposited in tissue, these autoantibodies incite a local capillaritis which manifests as rapidly progressive glomerulonephritis (GN) in 80 to 90% of patients, and with concurrent alveolar hemorrhage in ∼50%. A small proportion of cases may present with pulmonary disease in isolation. Serological testing for anti-GBM antibodies may facilitate rapid diagnosis, though renal biopsy is often required to confirm the presence of necrotizing or crescentic GN and linear deposition of autoantibody on the glomerular basement membrane. Alveolar hemorrhage may be evident clinically, or detected on imaging, pulmonary function testing, or bronchoscopy. Prompt treatment with plasmapheresis, cyclophosphamide, and steroids is usually indicated to remove pathogenic autoantibodies, to prevent their ongoing production, and to ameliorate end-organ inflammation. Alveolar hemorrhage is usually responsive to this treatment, and long-term respiratory sequelae are uncommon. Renal prognosis is more variable, though with aggressive treatment, independent renal function is maintained at 1 year in more than 80% of patients not requiring renal replacement therapy at presentation. Relapse in uncommon in anti-GBM disease, unless there is a concomitant antineutrophil cytoplasm antibody (present in 30–40%), in which case maintenance immunosuppression is recommended.

scholarly journals An uncommon cause of diffuse alveolar hemorrhage in a young male

2019 ◽  
Vol 89 (2) ◽  
Author(s):  
Anshul Mittal ◽  
Jagdish Chander Suri ◽  
Shibdas Chakrabarti ◽  
Pranav Ish
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Renal Involvement ◽  
Young Male ◽  
Diffuse Alveolar Hemorrhage ◽  
Alveolar Hemorrhage ◽  
Systemic Lupus ◽  
Threatening Condition ◽  
Life Threatening

It is uncommon for Systemic lupus erythematosus (SLE) to present with diffuse alveolar hemorrhage (DAH) as the initial presentation. To diagnose this in a young male with no renal involvement is further uncommon. We report a case of a 16-year-old boy, who presented with hemoptysis and was eventually diagnosed as DAH with underlying SLE. Treatment with steroids and immunosuppressant helped in rapid recovery from this potentially life-threatening condition. This case highlights the need of defining diagnostic criteria for SLE in patients presenting as DAH and formulating guidelines for treatment of the same, especially in absence of co-existing lupus nephritis.

A life-threatening complication of warfarin therapy in ED: diffuse alveolar hemorrhage

2014 ◽  
Vol 32 (6) ◽  
pp. 690.e3-690.e4 ◽  
Author(s):  
Emin Uysal ◽  
Erdem Çevik ◽  
Süleyman Solak ◽  
Yahya Ayhan Acar ◽  
Mustafa Yalimol
Keyword(s):  
Warfarin Therapy ◽  
Diffuse Alveolar Hemorrhage ◽  
Alveolar Hemorrhage ◽  
Life Threatening ◽  
Life Threatening Complication

Diffuse Alveolar Hemorrhage: a life-threatening adverse effect of vaping

2020 ◽  
Author(s):  
Dominika Ambrożej ◽  
Teresa Bielecka ◽  
Wojciech Feleszko ◽  
Katarzyna Krenke
Keyword(s):  
Adverse Effect ◽  
Diffuse Alveolar Hemorrhage ◽  
Alveolar Hemorrhage ◽  
Life Threatening

Langerin (CD207) Staining in Normal Pediatric Tissues, Reactive LymphNodes, and Childhood Histiocytic Disorders

2004 ◽  
Vol 7 (6) ◽  
pp. 607-614 ◽  
Author(s):  
Kudakwashe Chikwava ◽  
Ronald Jaffe
Keyword(s):  
Lymph Nodes ◽  
Staining Pattern ◽  
Granular Cell ◽  
Fixed Tissue ◽  
Cytoplasmic Staining ◽  
Immunohistochemical Markers ◽  
Rare Cells ◽  
Childhood Histiocytic Disorders ◽  
Cytoplasmic Staining Pattern ◽  
Skin Samples

Langerin is a recently identified lectin for which antibodies can be used as immunohistochemical markers of Langerhans cells (LCs). We describe the distribution of staining in autopsy pediatric tissues, dermatopathic and other reactive lymph nodes, and childhood histiocytic lesions using the 12D6 antibody (Novocastra). We also correlate CD1a (antibody O1O) staining to these factors. Langerin on epidermal LCs has a coarsely granular cell membrane and a cytoplasmic staining pattern that is always associated with CD1a expression. All 6 skin samples had Langerin+/CD1a+ LCs within the epidermis. Six of 8 thymuses showed single scattered dendritic-shaped cells in the medulla and rare cells within Hassall corpuscles that coexpressed Langerin and CD1a. Cortical thymocytes were CD1a+/Langerin-. Four of 8 livers examined showed a sinusoidal lining pattern of Langerin+/CD1a-. All 15 autopsy lymph nodes showed a similarly strong Langerin+/CD1a- sinus pattern of staining on fixed tissue elements, mostly in medullary sinuses. All 12 dermatopathic lymph nodes showed accumulation of Langerin+/CD1a+ cells in the pale paracortical nodules. All 24 instances of LC histiocytosis (LCH) were Langerin+/CD1a+. All 12 non-LCH histiocytic disorders are negative for Langerin in the histiocytes of interest. We conclude that Langerin is coexpressed with CD1a on LCs and LCH. Lymph node sinuses and hepatic sinusoids show Langerin+/CD1a-cells, indicating that, when used alone to confirm LCH infiltration, the 12D6 antibody should be used with caution. At other sites, its diagnostic accuracy is similar to that of CD1a.

scholarly journals Efficacy of Rituximab in a Systemic Lupus Erythematosus Patient Presenting with Diffuse Alveolar Hemorrhage

2017 ◽  
Vol 2017 ◽  
pp. 1-7 ◽  
Author(s):  
Gabriela Montes-Rivera ◽  
Grissel Ríos ◽  
Luis M. Vilá
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Therapeutic Option ◽  
Acute Onset ◽  
Diffuse Alveolar Hemorrhage ◽  
Alveolar Hemorrhage ◽  
High Dose ◽  
Systemic Lupus ◽  
Effective Therapeutic Option ◽  
Life Threatening

Diffuse alveolar hemorrhage (DAH) is a life-threatening complication of systemic lupus erythematosus (SLE). Although infrequent, its mortality is very high. While there are no established therapeutic guidelines, DAH has been traditionally managed with high-dose intravenous (IV) corticosteroids, cyclophosphamide, and plasma exchange. The efficacy of alternative therapies such as rituximab has been described only in a few cases. Herein, we report a 25-year-old Hispanic man who presented with acute-onset SLE manifested by polyarthralgia, nephritis, seizures, pancytopenia, severe hypocomplementemia, and elevated anti-dsDNA antibodies. His disease course was complicated by DAH. His condition was refractory to high-dose intravenous (IV) methylprednisolone pulses, IV cyclophosphamide, and plasmapheresis. Given the lack of clinical response, he was started on IV rituximab 375 mg/m2 weekly for a total of four courses. He rapidly improved after the first two doses. Over the next seven months, he did not present recurrent pulmonary symptoms. Follow-up chest computed tomography did not show residual abnormalities. This case, together with other reports, suggests that rituximab is an effective therapeutic option for DAH in SLE.

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