scholarly journals Microscopic Polyangitis in Pediatric Systemic Lupus Erythematosus: A Unique Presentation of Pulmonary - Renal Syndrome and Case Report of an Overlap Syndrome

2020 ◽  
Author(s):  
Chen-xing Zhang ◽  
Lei Yin ◽  
You-ying Mao ◽  
Zheng-yu Zhou ◽  
He Qin ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Systemic Vasculitis ◽  
Rapidly Progressive Glomerulonephritis ◽  
Overlap Syndrome ◽  
Systemic Lupus ◽  
Pediatric Systemic Lupus Erythematosus ◽  
Pulmonary Renal Syndrome ◽  
Pulmonary Alveolar Hemorrhage ◽  
Lupus Glomerulonephritis

Abstract Background: Secondary vasculitis is encountered in about one third of all cases of systemic lupus erythematosus (SLE). Skin is most commonly involved in lupus-related small vasculitis. Although antineutrophil cytoplasmic autoantibodies (ANCA) associated vasculitis (AAV) is relatively uncommon, it can be the most dangerous manifestation associated with high mortality. SLE and AAV are separate diseases with different pathophysiologies and an overlap syndrome has only been reported few times in previous literature. Case Presentation: We present a unique case of a pediatric patient of pulmonary-renal syndrome, presenting with pulmonary alveolar hemorrhage and rapidly progressive glomerulonephritis. Serological and biopsy findings were suggestive of SLE and AAV occurring simultaneously. Renal biopsy demonstrated necrotizing and crescentic glomerulonephritis, superimposed on diffuse segmental proliferative lupus glomerulonephritis class IV. Conclusions: Presentations of autoimmune diseases and vasculitis can be multi-systemic. Considering overlap syndromes, especially in patients with underlying connective tissue disease or systemic vasculitis, is vital for prompt therapy and prevention of morbidity in this population.

scholarly journals Involvement of neutrophil extracellular traps in the pathogenesis of glomerulonephritis in a case of systemic lupus erythematosus and antineutrophil cytoplasmic antibody-associated vasculitis overlap syndrome

2022 ◽  
Author(s):  
Arisa Senda ◽  
Ryutaro Sasai ◽  
Kurumi Kato ◽  
Yuka Nishibata ◽  
Sakiko Masuda ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Healthy Subjects ◽  
Renal Damage ◽  
Rapidly Progressive Glomerulonephritis ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Neutrophil Extracellular Traps ◽  
Overlap Syndrome ◽  
Systemic Lupus ◽  
Extracellular Traps

AbstractSystemic lupus erythematosus (SLE) and antineutrophil cytoplasmic antibody-associated vasculitis (AAV) are autoimmune diseases that often cause rapidly progressive glomerulonephritis, with neutrophil extracellular traps (NETs) involved in their pathogenesis. However, the involvement of NETs in the renal damage caused by SLE/AAV overlap syndrome has not been clarified yet. In this study, we detected renal deposition of NETs in a patient with SLE/AAV overlap syndrome. In addition, a significantly increased level of NET-inducing activity was observed in the patient’s serum, which improved with treatment. On the other hand, a markedly lower level of NET degradation was observed in the patient’s serum as compared to healthy subjects’ sera, without any posttreatment changes. These findings suggest that NETs may play a role in the pathogenesis of renal injury associated with SLE/AAV overlap syndrome.

Fulminant systemic vasculitis in systemic lupus erythematosus. Case report and review of the literature

Lupus ◽  
2014 ◽  
Vol 23 (13) ◽  
pp. 1426-1429 ◽  
Author(s):  
G Medina ◽  
D González-Pérez ◽  
C Vázquez-Juárez ◽  
M Sánchez-Uribe ◽  
M A Saavedra ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Systemic Vasculitis ◽  
Clinical Manifestations ◽  
Rapidly Progressive Glomerulonephritis ◽  
Lower Limbs ◽  
Systemic Lupus ◽  
Life Threatening ◽  
History Of ◽  
Level Hospital

Vasculitis in systemic lupus erythematosus (SLE) has a broad spectrum of clinical manifestations from cutaneous to visceral involvement and its prognosis ranges from mild to life-threatening. We report the case of a previously healthy 17-year-old woman with eight months' history of arthralgias and myalgias. Subsequently, she developed facial and lower limbs edema, and hair loss. Two weeks before admission to a secondary level hospital, she developed fever up to 40 ℃ followed by abdominal pain, rectal bleeding, hematemesis and blisters on both legs, reason for which she was hospitalized. With active bullous SLE with rapidly progressive glomerulonephritis suspected, she was treated with methylprednisolone pulses without response. After one week of treatment, she was transferred to a tertiary level hospital. On admission she presented acute arterial insufficiency of the lower extremities, respiratory failure with apnea, metabolic acidosis and shock; six hours later she died. Autopsy findings showed active diffuse lupus nephritis and diffuse systemic vasculitis that involved vessels from the skin, brain, myocardium, spleen, iliac and renal arteries. In addition, serositis of the small intestine and colon, acute and chronic pericarditis, pericardial effusion and myocarditis were found. Immunologic tests confirmed SLE diagnosis. In this case the fulminant course was the result of SLE high disease activity, visceral vasculitis of several organs and late diagnosis, referral and treatment. Early diagnosis, and opportune referral to the rheumatologist for intensive treatment can improve the outlook in these patients.

Rapidly progressive glomerulonephritis as presenting feature of systemic lupus erythematosus in a Bangladeshi male patient: a rare case report

2020 ◽  
Vol 10 (2) ◽  
pp. 137-138
Author(s):  
Samiha Haque ◽  
Ishrat Jahan ◽  
Tufayel Ahmed Chowdhury ◽  
Muhammad Abdur Rahim ◽  
Mehruba Alam Ananna ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Venous Pressure ◽  
Rapidly Progressive Glomerulonephritis ◽  
Initial Response ◽  
Systemic Lupus ◽  
Renal Manifestation ◽  
Rare Case Report ◽  
Diagnostic Work ◽  
Work Up

Rapidly progressive glomerulonephritis is one of the most dramatic and tragic presentations of lupus nephritis (LN) or renal manifestation of systemic lupus erythematosus (SLE). A 35-year-old Bangladeshi gentleman presented with worsening oedema, scanty, high colored, frothy urine and deteriorating renal function. He had puffy face, anaemia, oedema, normal jugular venous pressure (JVP), high blood pressure (150/90 mm Hg), ascites and bilateral pleural effusions. Diagnostic work-up confirmed SLE with class IV LN. His initial response to specific therapy showed improvement Birdem Med J 2020; 10(2): 137-138

LUPUS NEPHRITE WITH FAST - PROGRESSING CURRENT: CASE REPORT

Vestnik ◽  
2021 ◽  
pp. 143-146
Author(s):  
Б.Г. Султанова ◽  
С.Б. Бодесова ◽  
А.Т. Ибрашева ◽  
Б.С. Мусабаев ◽  
Д.Ш. Бетирова ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Case Report ◽  
Lupus Erythematosus ◽  
Clinical Laboratory ◽  
Rapidly Progressive Glomerulonephritis ◽  
Diagnostic Methods ◽  
Symptomatic Therapy ◽  
Systemic Lupus ◽  
Current Case ◽  
Complex Therapy

В статье описан «неклассический» случай, редко встречающаяся форма заболевания системной красной волчанкой без типичного поражения кожи с проявлением быстропрогрессирующего гломерулонефрита, с поражением тазобедренного сустава, выраженным болевым синдромом у юноши. С применением новых инновационных методов диагностики (непрямая иммунофлюоресценция на анализаторе AKLIDES), что позволило провести своевременно комплексную терапию включая в себя патогенетическую, эфферентную (гемодиализ, плазмаферез), тем самым получен хороший клинический эффект. This article describes a non- racial case of systemic lupus erythematosus with the manifestation of a rapidly progressive glomerulonephritis, hip joint lesion, a pronounced painful syndrom, without dermal manifestations. By the use of new diagnostic methods (immunofluorescence), timely complex therapy: pathogenetic, efferent therapy, symptomatic therapy, it is possible to obtain a fairly good clinical - laboratory- instrumental result.

Sign in / Sign up

Export Citation Format

Share Document