scholarly journals Shrinking lung syndrome treated with rituximab in pediatric systemic lupus erythematosus: a case report and review of the literature

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Chelsea DeCoste ◽  
Dimas Mateos-Corral ◽  
Bianca Lang
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lung Function ◽  
Lupus Erythematosus ◽  
Rare Complication ◽  
Optimal Treatment ◽  
Normal Lung ◽  
Systemic Lupus ◽  
Lung Volumes ◽  
Malar Rash ◽  
Normal Lung Function

Abstract Background Shrinking lung syndrome (SLS), a rare complication of systemic lupus erythematosus (SLE) characterized by dyspnea, low lung volumes, and a restrictive pattern on pulmonary function tests (PFTs), has only been reported in a few children. Given the rarity of SLS there is a paucity of literature regarding its optimal treatment. Outcomes are variable, with case reports documenting some improvement in most patients treated with corticosteroids, with or without additional immunosuppressive agents. However, most reported patients did not recover normal lung function. We report full recovery of a child with SLE and SLS following treatment with rituximab and review the current literature. Case presentation An 11-year-old boy presented with a malar rash, myositis, arthritis, oral ulcers, leukopenia, anemia, positive lupus autoantibodies and Class II nephritis. He was diagnosed with SLE and treated with corticosteroids, hydroxychloroquine, azathioprine, and subsequently mycophenolate with symptom resolution. At age 14, his SLE flared coincident with a viral chest infection. He presented with a malar rash, polyarthritis, increased proteinuria and pleuritis which all improved with corticosteroids and ongoing treatment with mycophenolate. Six weeks later he presented with severe dyspnea, markedly decreased lung volumes, but otherwise normal chest X-ray (CXR) and high-resolution chest computed tomography (HRCT). He was found to have severely restricted PFTs (FEV1 27%, FVC 29%; TLC 43%). After additional investigations including echocardiography, pulmonary CT angiography, and diaphragmatic fluoroscopy, he was diagnosed with SLS and treated with rituximab and methylprednisolone. At 1 month his symptoms had improved, but he still had dyspnea with exertion and severely restricted PFTs. At 6 months his FVC and TLC had improved to 51 and 57% respectively, and were 83 and 94% respectively at 4 years. He had returned to all baseline activities, including competitive hockey. Conclusions Although extremely rare, it is important to recognize SLS as a possible cause of dyspnea and chest pain in a child with SLE. Optimal treatment strategies are unknown. This is the second reported case of a child treated with rituximab for SLS who recovered normal lung function. International lupus registries should carefully document the occurrence, treatment and outcome of patients with SLS to help determine the optimal treatment for this rare complication.

scholarly journals Differential diagnosis of a thyroid mass, facial malar rash and ptosis on the flora in the primavera by Sandro Botticelli (1445–1510)

2021 ◽  
Author(s):  
H. Ashrafian
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Systemic Lupus ◽  
Bilateral Ptosis ◽  
Malar Rash ◽  
Autoimmune Syndrome ◽  
Thyroid Mass ◽  
Multiple Autoimmune Syndrome ◽  
Sandro Botticelli ◽  
Renaissance Artist

Abstract Purpose The Primavera is considered amongst the greatest and controversial artistic masterpieces worldwide painted by renaissance artist Sandro Botticelli. The aim was to identify any underlying medical foundations for the painting. Methods Observational study. Results The painting reveals, a ‘butterfly’ malar rash, bilateral ptosis and a clear neck swelling consistent with a goitre in the figure of Flora. This could be explained by concomitant Graves’ disease and systemic lupus erythematosus, or other presentations of multiple autoimmune syndrome. Conclusion These findings highlight the likely presentation of the earliest pictorial depictions of thyroid disease with systemic lupus erythematosus and emphasize the exactitude of depiction demonstrated by Botticelli in renaissance era.

scholarly journals Cell-mediated amegakaryocytic thrombocytopenia associated with systemic lupus erythematosus

Blood ◽  
1986 ◽  
Vol 67 (2) ◽  
pp. 479-483
Author(s):  
T Nagasawa ◽  
T Sakurai ◽  
H Kashiwagi ◽  
T Abe
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Bone Marrow ◽  
T Cells ◽  
Lupus Erythematosus ◽  
Culture System ◽  
Bone Marrow Cells ◽  
Rare Complication ◽  
Systemic Lupus ◽  
Marrow Cells

We studied a patient with a rare complication of amegakaryocytic thrombocytopenia (AMT) associated with systemic lupus erythematosus (SLE). To investigate the underlying pathogenesis of AMT, the effects of peripheral blood T cells and serum on human megakaryocyte progenitor cells were studied using in vitro coculture techniques. Mononuclear bone marrow cells (2 X 10(5) from normal donors produced 33.6 +/- 8.8 (n = 10) colony-forming unit-megakaryocytes (CFU-M) in our plasma clot system. When 2 X 10(5) of the patient's T cells were added to the culture system, the number of CFU-M decreased to only 3.5 +/- 0.6/2 X 10(5) bone marrow cells. No evidence of inhibitory effects was found by the addition of the patient's serum and complement to the culture system. The T cells stored at -80 degrees C on admission were also capable of suppressing autologous CFU-M after recovery from AMT. These results indicate that in vitro suppression of CFU-M from allogenic and autologous bone marrow cells by this patient's T cells provides an explanation for the pathogenesis of AMT associated with SLE.

SAT0217 PERFORMANCE OF ACR/EULAR 2019, SLICC 2012 AND ACR 1997 CLASSIFICATION CRITERIA IN A COHORT OF SYSTEMIC LUPUS ERYTHEMATOSUS PATIENTS WITH LONGSTANDING DISEASE

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1051-1052
Author(s):  
D. Lobo Prat ◽  
B. Magallares ◽  
I. Castellví ◽  
H. Park ◽  
P. Moya ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Statistical Significance ◽  
Renal Involvement ◽  
High Sensitivity ◽  
Classification Criteria ◽  
Average Score ◽  
Systemic Lupus ◽  
Malar Rash ◽  
Oral Ulcers

Background:Systemic lupus erythematosus (SLE) is an autoimmune disease with variable clinical features and a complex physiopathology. In 2019, EULAR and ACR have jointly developed new classification criteria with both high sensitivity and specificity. These criteria have the particularity of including the presence of ANA as an obligatory entry criterion and the existence of clinical and immunological domains with weighted scores.Objectives:To evaluate the performance and characteristics of the ACR/EULAR 2019, SLICC 2012 and ACR 1997 classification criteria in a cohort of SLE patients with longstanding disease.Methods:Descriptive observational study that enrolled a cohort of SLE patients with longstanding disease followed in a tertiary level hospital. Demographic and clinical data were gathered along with the fulfillment of classification criteria. The sensitivity of each classification criteria and the statistically significant associations between criteria fulfillment and clinical and immunological data were calculated. Statistical analyses were performed using the Chi2, T-student and ANOVA tests. Statistical significance was assumed in p values <0.05.Results:A total of 79 patients (88.6% women) with a mean age of 51.8±14 years, disease duration of 15.2±11.5 years and SLEDAI of 2.65±2.1 were included. The sensitivity of the different classification criteria was 51.9% for ACR 1997, 87.3% for SLICC 2012 and 86.1% for ACR/EULAR 2019 (Table 1).Table 1.Sensitivity and average scores.ACR/EULAR 2019SLICC 2012ACR 1997Sensitivity (%)86.187.351.9Average score of patients classified as SLE(±SD)18.6±5.85.3±1.45±0.9Average score of patients NOT classified as SLE(±SD)6.1±2.52.8±0.42.8±0.851.9% of patients met all three classification criteria, 29.1% met SLICC 2012 and ACR/EULAR 2019, 5% only met SLICC 2012 and 3.7% exclusively met ACR/EULAR 2019. 11.4% of patients did not meet any classification criteria and were characterized by having a low SLEDAI (0.6±0.9) and fulfilling only skin domains (alopecia or oral ulcers), antiphospholipid antibodies or hypocomplementemia.Statistically significant associations were found between meeting ACR/EULAR 2019 classification criteria and the presence of low C3 and C4 (p<0.04), DNA (p<0.001), lupus nephritis III-IV (p<0.05) and arthritis (p<0.001), highlighting that all patients with arthritis met these criteria.In the SLICC 2012 evaluation, significant associations were found between meeting these criteria and the presence of arthritis (p<0.01), renal involvement (p<0.04), leukopenia/lymphopenia (p=0.05), DNA (p<0.03) and hypocomplementemia (p=0.02).Fullfilment of ACR 1997 was associated to the presence of malar rash (p<0.001), discoid lupus (p<0.05), photosensitivity (p<0.001) and oral ulcers (p<0.04), as well as arthritis (p<0.001), serositis (p=0.02), renal (p<0.05) and hematologic (p=0.05) involvement.The Kappa concordance coefficient among classification criteria is detailed in Table 2.Table 2.Kappa concordance coefficient.ACR/EULAR 2019 - SLICC 2012ACR/EULAR 2019 - ACR 1997SLICC 2012 - ACR 1997Kappa concordance coefficient0.610.270.30Conclusion:The ACR/EULAR 2019 classification criteria maintain a high sensitivity similar to the SLICC 2012 in SLE patients with longstanding disease, both of which are much higher than ACR 1997. Patients with serological, articular or renal involvement are more likely to meet SLICC 2012 or ACR/EULAR 2019 criteria. It is noteworthy the relevance of dermatological manifestations in ACR1997 classification criteria against the increased weight that a better understanding of SLE physiopathology has provided to analytic and immunological criteria in the subsequent classification criteria.Disclosure of Interests:David Lobo Prat: None declared, Berta Magallares: None declared, Ivan Castellví Consultant of: Boehringer Ingelheim, Actelion, Kern Pharma, Speakers bureau: Boehringer Ingelheim, Actelion, Bristol-Myers Squibb, Roche, HyeSang Park: None declared, Patricia Moya: None declared, Ignasi Gich: None declared, Ana Laiz: None declared, Cesar Díaz-Torné: None declared, Ana Milena Millán Arciniegas: None declared, Susana P. Fernandez-Sanchez: None declared, Hector Corominas: None declared

Lupus Eritematosus Sistemik pada Pria

2021 ◽  
Vol 3 (2) ◽  
pp. 37-42
Author(s):  
Harry Andrean ◽  
Raveinal Raveinal
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Clinical Course ◽  
Multiple Organ ◽  
Systemic Lupus ◽  
Malar Rash ◽  
Mouth Ulcer ◽  
Pulse Dose ◽  
Aggressive Clinical Course ◽  
Complex Autoimmune Disease

Introduction: Systemic lupus erythematosus (SLE) is a complex autoimmune disease characterized by presence of nucleus autoantibody and affected multiple organ. Systemic lupus erythematosus is more common in women than men with ratio 2:1 to 15:1. Men with SLE often have a more aggressive clinical course, lead to a poorer prognosis compared with women with SLE. Case Report: A man, 29 years old came to hospital with main complain joint pain increased since 1 week ago, accompanied with red spot on face, trunk, hands, foot, and back, hair loss, swollen leg, mouth ulcer, and fatique. Malar rash and discoid rash were identified from physical examination. From laboratorium, ANA profile was positive for RNP/Sm, Sm, dsDNA, and histone. Skin biopsy showed a lupus discoid. Conclusion: The patient was treated with pulse-dose methylprednisolone for 3 days and showed a good response clinically.

scholarly journals Primary Coronary Sinus Thrombosis in Secondary Antiphospholipid Antibody Syndrome

2011 ◽  
Vol 2 (2) ◽  
pp. 102-104
Author(s):  
Joseph Theodore ◽  
P. Chitrambalam ◽  
K. Pradeep ◽  
S. Viswakumar
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Coronary Sinus ◽  
Lupus Erythematosus ◽  
Sinus Thrombosis ◽  
Rare Complication ◽  
Antiphospholipid Antibody ◽  
Antiphospholipid Antibody Syndrome ◽  
Systemic Lupus ◽  
Inflammatory Autoimmune Disease ◽  
Intracardiac Thrombosis

Antiphospholipid antibody syndrome (APLA) is a non-inflammatory autoimmune disease characterised by spontaneous abortion, thrombocytopenia and thrombosis (arterial and venous). Intracardiac thrombosis is a rare complication of APLA, but coronary sinus thrombosis in APLA has hitherto not been reported. We recently treated a young woman with secondary APLA and systemic lupus erythematosus in whom coronary sinus thrombosis was detected in association with recurrent pulmonary embolism. Key Words: intracardiac thrombosis; antiphospholipid antibody syndrome; systemic lupus erythematosus; coronary sinus thrombosis DOI: http://dx.doi.org/10.3126/ajms.v2i2.3885 Asian Journal of Medical Sciences 2 (2011) 102-104

scholarly journals Recurrent Afebrile Episodes of Spontaneous Pneumothorax in a Child with Well Controlled Systemic Lupus Erythematosus Disease

2016 ◽  
Vol 36 (1) ◽  
pp. 94-96
Author(s):  
Sudhir Mehta
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Immunosuppressive Therapy ◽  
Lupus Erythematosus ◽  
Spontaneous Pneumothorax ◽  
Rare Complication ◽  
Autoimmune Disorder ◽  
Pulmonary Involvement ◽  
Systemic Lupus Erythematosus Disease ◽  
Systemic Lupus ◽  
Childhood Sle

Systemic Lupus Erythematosus is an autoimmune disorder with multiple system involvement. Despite its pleuro-pulmonary involvement, recurrent spontaneous pneumothoraces are rare complication of childhood SLE. We report a 12 year old girl having SLE with nephritis, who developed recurrent episodes of spontaneous pneumothoraces. Surgical management with prolonged evacuation and aggressive immunosuppressive therapy improves the outcome for these patients. J Nepal Paediatr Soc 2016;36(1):94-96.

scholarly journals Immune Thrombocytopenic Purpura as Initial Presentation of Paediatric SLE: A Case Report

2020 ◽  
Vol 59 (233) ◽  
Author(s):  
Anjila Ghimire
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Platelet Count ◽  
Case Report ◽  
Lupus Erythematosus ◽  
Female Child ◽  
Connective Tissue Disorder ◽  
Systemic Lupus ◽  
Thrombocytopenic Purpura ◽  
Malar Rash ◽  
Mucosal Bleeding

The systemic lupus erythematosus (SLE) is a connective tissue disorder with variable presentationsin children. The usual presentation includes arthritis, malar rash, nephritis, hemolytic anemia, andfever. Isolated hematologic abnormality as the only presentation of SLE is rare. Here is a case reportof a female child presented to us with superficial and mucosal bleeding with isolated low plateletcount and anemia in proportion to blood loss. When platelet count didn’t go up despite appropriatetreatment in lines of ITP, further investigations were done, diagnosis of SLE was established, andmanagement was done accordingly.

scholarly journals Pulmonary Hypertension in Systemic Lupus Erythematosus with Raynaud’s Phenomenon: Case Report

2021 ◽  
Vol 13 (1) ◽  
pp. 476-482
Author(s):  
Agnes Dina Irene Dorithy Zagoto ◽  
Ayu Paramaiswari
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Pulmonary Hypertension ◽  
Lupus Erythematosus ◽  
Rare Complication ◽  
Raynaud’S Phenomenon ◽  
Signs And Symptoms ◽  
Right Heart Failure ◽  
Raynaud's Phenomenon ◽  
Systemic Lupus ◽  
Multiple Organs

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that affects multiple organs such as skin, joints, kidneys, heart, lungs, nervous system and blood. Pulmonary hypertension is a rare complication of SLE.1 Systemic lupus erythematosus associated with pulmonary hypertension was defined as an increase in the average pressure in the pulmonary artery at rest ? 25 mmHg with pulmonary capillary wedge pressure ?15 mm Hg and an increase in pulmonary vascular resistance.2 The prevalence of pulmonary hypertension in SLE approximately 0.5 to 17.5%. Predictors factors of the occurrence of pulmonary hypertension in LES is Raynaud's phenomenon, anti-U1RNP antibody, and antibody positive anticardiolipin.3 A woman aged 37 years came with a chief complaint of pain in the fingers and toes with black-colored wounds felt since 6 months before admission. From the anamnesis, physical examination, support to meet 5 of the classification criteria for systemic lupus erythematosus based on the 1997 ACR criteria which includes manifestations of arthritis, mucocutaneous, serositis, lupus antiokoagulan, and ANA IF positive. In these patients also found the typical signs and symptoms of Raynaud's phenomenon which leads to the symptoms of pain in the fingers of both hands when exposed to cold and pale to red when heated and has been confirmed from the results of arteriography. From the results of echocardiography reveal any pulmonary hypertension. This patient was treated with steroids, immunosuppressants and antiplatelet. The case was removed because of pulmonary hypertension is a complication LES rare and necessary sharpness in diagnosis. Patients with pulmonary hypertension of unknown or untreated can become a progressive right heart failure and lead to death.3

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