scholarly journals Lupus Anticoagulant Hypoprothrombinemia Syndrome Associated with Bilateral Adrenal Hemorrhage in a Child: Early Diagnosis and Intervention

2020 ◽  
Author(s):  
Atsushi Sakamoto ◽  
Masao Ogura ◽  
Atsushi Hattori ◽  
Kinji Tada ◽  
Reiko Horikawa ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Lupus Anticoagulant ◽  
Early Recognition ◽  
Clinical Manifestations ◽  
Adrenal Hemorrhage ◽  
Screening Tests ◽  
Adrenal Failure ◽  
Systemic Lupus ◽  
Life Threatening

Abstract Background: Lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS) is characterized by bleeding and thrombosis in patients, usually associated with autoimmunity or infections. Pediatric LAHPS exhibits various degrees of bleeding, ranging from mild to severe; however, adrenal hemorrhage due to LAHPS and its long-term clinical course have not been sufficiently described. Case presentation: A 9‐year‐old boy presented with prolonged abdominal pain and abnormal coagulation screening tests. The laboratory tests showed prolonged activated partial thromboplastin time and subsequently revealed the presence of lupus anticoagulant, anti-nuclear antibodies, and hypoprothrombinemia, leading to diagnosis of LAHPS. An enhanced computed tomogram demonstrated nodular lesions in the adrenal glands bilaterally, suggestive of adrenal hemorrhage. Laboratory and clinical manifestations exhibited life-threatening adrenal insufficiency that required hydrocortisone administration. The patient developed systemic lupus erythematosus, diagnosed 12 months later. Conclusions: The patient with LAHPS developed rare adrenal failure due to adrenal hemorrhage, a life-threatening event that should be recognized and treated early. In our case, renal dysfunction was also observed when systemic lupus erythematosus was diagnosed one year after LAHPS. Our case emphasizes that early recognition of adrenal failure and careful observation for patients with autoantibodies are required.

scholarly journals Lupus anticoagulant hypoprothrombinemia syndrome associated with bilateral adrenal haemorrhage in a child: early diagnosis and intervention

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Atsushi Sakamoto ◽  
Masao Ogura ◽  
Atsushi Hattori ◽  
Kinji Tada ◽  
Reiko Horikawa ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Lupus Anticoagulant ◽  
Clinical Manifestations ◽  
Screening Tests ◽  
Adrenal Failure ◽  
Adrenal Haemorrhage ◽  
Systemic Lupus ◽  
Life Threatening

Abstract Background Lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS) is characterized by bleeding and thrombosis in patients with autoimmune diseases or infections. Paediatric LAHPS exhibits various degrees of bleeding, ranging from mild to severe; however, adrenal haemorrhage due to LAHPS and its long-term clinical course have not been sufficiently described. Case presentation A 9-year-old boy presented with prolonged abdominal pain and abnormal coagulation screening tests. The laboratory tests showed prolonged activated partial thromboplastin time and subsequently revealed the presence of lupus anticoagulant, anti-nuclear antibodies, and hypoprothrombinemia, leading to diagnosis of LAHPS. An enhanced computed tomogram demonstrated nodular lesions in the adrenal glands bilaterally, suggestive of adrenal haemorrhage. Laboratory and clinical manifestations exhibited life-threatening adrenal insufficiency that required hydrocortisone administration. The patient developed systemic lupus erythematosus, diagnosed 12 months later. Conclusions This patient with LAHPS developed rare adrenal failure due to adrenal haemorrhage, a life-threatening event that should be recognized and treated early. In our case, renal dysfunction was also observed when systemic lupus erythematosus was diagnosed 1 year after LAHPS. Our case emphasizes that early recognition of adrenal failure and careful long-term observation is required in patients with autoantibodies.

Systemic lupus erythematosus—clinical features and aetiopathogenesis

2013 ◽  
pp. 923-937
Author(s):  
Caroline Gordon
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Differential Diagnosis ◽  
Autoimmune Disease ◽  
Clinical Features ◽  
Lupus Erythematosus ◽  
Immune Complexes ◽  
Clinical Manifestations ◽  
Systemic Lupus ◽  
Life Threatening ◽  
Symptoms And Signs

Systemic lupus erythematosus (SLE or lupus) is a multisystem, autoimmune disease associated with the formation of autoantibodies that form pathological immune complexes and activate a number of inflammatory pathways. The disease is characterized by remissions and relapses (flares) that can present with a variety of clinical manifestations. The symptoms and signs may range from mild features that can be treated easily to organ and even life threatening manifestations requiring potent immunosuppression. This chapter will review the epidemiology and pathology of lupus, then the clinical features including differential diagnosis and investigation of adult patients with SLE. Finally the classification, diagnosis, monitoring and outcome of lupus patients will be discussed.

scholarly journals Antiphospholipid syndrome accompanying systemic lupus erythematosus

2002 ◽  
Vol 55 (3-4) ◽  
pp. 89-96 ◽  
Author(s):  
Gorana Mitic
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Antiphospholipid Antibodies ◽  
Lupus Anticoagulant ◽  
Clinical Manifestations ◽  
Spontaneous Abortions ◽  
Systemic Lupus ◽  
Recurrent Spontaneous Abortions ◽  
Thrombotic Complications

The aim of the study was the assessment of the prevalence of antiphospholipid syndrome (APS) in patients with systemic lupus erythematosus (SLE). 72 patients with SLE had been investigated, 66 females and six males, aged 17 to 70 years, average 37,03. The presence of APA was determined using both ELISA assay for antiphospholipid antibodies ASSERACHROM APA by Diagnostica Stago and clotting tests for lupus anticoagulant: activated partial thromboplastin time (aPTT), tissue thromboplastin inhibition test (TTI) and dilute Russell viper venom time (dRVVT). Antiphospholipid antibodies have been found in 24 patients (33.44%), 10 of them were. with positive lupus anticoagulant tests, 6 of them were with positive ELISA test, while 8 of them had positive coagulation and immunological tests. Clinical manifestations that could be related to antiphospholipid syndrome were present in 22 patients (30.5%). The most common were thrombotic complications in 16 patients (22.25), recurrent spontaneous abortions in 7 patients (9.7%) and thrombocytopenia in 1 patient (1.39%). Presence of antiphospholipid syndrome was determined in 15 patients (20.83%). We can conclude that there is a significant correlation between presence of antiphospholipid antibodies and both thrombotic events and recurrent spontaneous abortions in SLE patients. Occurrence of thrombotic complications is in direct correlation with the level of antiphospholipid antibodies.

Systemic lupus erythematosus—clinical features and aetiopathogenesis

2013 ◽  
Author(s):  
Caroline Gordon
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Differential Diagnosis ◽  
Autoimmune Disease ◽  
Clinical Features ◽  
Lupus Erythematosus ◽  
Immune Complexes ◽  
Clinical Manifestations ◽  
Systemic Lupus ◽  
Life Threatening ◽  
Symptoms And Signs

Systemic lupus erythematosus (SLE or lupus) is a multisystem, autoimmune disease associated with the formation of autoantibodies that form pathological immune complexes and activate a number of inflammatory pathways. The disease is characterized by remissions and relapses (flares) that can present with a variety of clinical manifestations. The symptoms and signs may range from mild features that can be treated easily to organ and even life threatening manifestations requiring potent immunosuppression. This chapter will review the epidemiology and pathology of lupus, then the clinical features including differential diagnosis and investigation of adult patients with SLE. Finally the classification, diagnosis, monitoring and outcome of lupus patients will be discussed.

scholarly journals AB0308 COGNITIVE IMPAIRMENT IN SYSTEMIC LUPUS ERYTHEMATOSUS AND ANTIPHOSPHOLIPID SYNDROME PATIENTS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1180.1-1180
Author(s):  
A. Borisova ◽  
T. Lisitsyna ◽  
D. Veltishchev ◽  
T. Reshetnyak ◽  
O. Seravina ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Cognitive Impairment ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Lupus Anticoagulant ◽  
Depressive Disorders ◽  
Clinical Manifestations ◽  
Structured Interview ◽  
Livedo Reticularis ◽  
Systemic Lupus

Background:Cognitive impairment (CI) in systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS) patients have been poorly described and recognized.Objectives:to describe the rates and spectrum of CI in primary (PAPS) and secondary to systemic lupus erythematosus (SLE) (SAPS) APS patientsMethods:113 patients (70 with APS (37 – PAPS, 33 – SAPS) and 43 - SLE without APS), 89 (78,8%) – women, were consecutively enrolled in the study. The mean (M±SD) age was 37,9±11,9 years. SLE activity was measured by SLEDAI scale. Mental disorders (MD) were diagnosed by psychiatrist in accordance with ICI-10 in semi-structured interview. CI were diagnosed with psychology and neuropsychology methodsResults:CI of varying severity were found in 105 (92,9%) patients: 62,9% - mild, 23,8% - moderate and 13,3% - severe. Severe and moderate CI were more associated with APS (48,6% in PAPS and 39,5% in SAPS vs 18,6% in SLE, p=0,004 and p=0,04, accordingly). CI were predominantly organic origin in all patients, but vascular dementia was detected only among patients with APS (10,8% of PAPS and 3,03% of SAPS patients). There was no association of CI with clinical manifestations and SLE activity. In patients with PAPS CI was associated with stroke, livedo reticularis and lupus anticoagulant positivity. In 84 patients (74,3%) CI were also specifically bounded to MD. Current MD were detected in 100 (88,5%) patients: schizotypal disorder was found in 10 (8,85%) patients and was associated with PAPS (13,5% vs 9,09% in SAPS and 4,65% in SLE); anxiety-depressive spectrum disorders (ADDs) - in 95 (84,1%) (chronic and recurrent depression prevailed 37 (32,7%) and 42 (37,2%) resp.); the structure of MDs in accordance with ICI-10 differed slightly between groups, but no statistically significant differences were obtained.Conclusion:сognitive impairment, mainly of an organic type, are characteristic of most patients with SLE and APS. The significant associations of сognitive impairment with clinical manifestations and activity of SLE were not identified, but patients with сognitive impairments were more likely to have anxiety and depressive disorders, strokes, livedo reticularis and lupus anticoagulant positivityDisclosure of Interests:None declared

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.

scholarly journals Respiratory Manifestations in Systemic Lupus Erythematosus

2021 ◽  
Vol 14 (3) ◽  
pp. 276
Author(s):  
Salvatore Di Bartolomeo ◽  
Alessia Alunno ◽  
Francesco Carubbi
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Wide Spectrum ◽  
Clinical Manifestations ◽  
Specific Pattern ◽  
Systemic Autoimmune Disease ◽  
Therapeutic Approaches ◽  
Systemic Lupus ◽  
Respiratory Involvement ◽  
Life Threatening

Systemic lupus erythematosus (SLE) is a chronic systemic autoimmune disease characterized by a wide spectrum of clinical manifestations. The respiratory system can be involved in up to 50–70% of patients and be the presenting manifestation of the disease in 4–5% of cases. Every part of the respiratory part can be involved, and the severity can vary from mild self-limiting to life threatening forms. Respiratory involvement can be primary (caused by SLE itself) or secondary (e.g., infections or drug toxicity), acute or chronic. The course, treatment and prognosis vary greatly depending on the specific pattern of the disease. This review article aims at providing an overview of respiratory manifestations in SLE along with an update about therapeutic approaches including novel biologic therapies.

scholarly journals Systemic vasculitis in a patient with rhupus syndrome

Reumatismo ◽  
2016 ◽  
Vol 67 (4) ◽  
pp. 161 ◽  
Author(s):  
O. Zengin ◽  
M. E. Onder ◽  
M. A. Sarica ◽  
I. H. Turkbeyler ◽  
G. Kimyon ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Systemic Lupus Erythematosus ◽  
Young Women ◽  
Lupus Erythematosus ◽  
Systemic Vasculitis ◽  
Clinical Manifestations ◽  
Organ Involvement ◽  
Systemic Lupus ◽  
Rare Syndrome ◽  
Life Threatening

Rhupus is a rare syndrome characterized by overlap of rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE). Previous reports mentioned that rhupus patients have prominent RA associated clinical manifestations and only mild organic damage related to SLE. Progressive or life-threatening manifestations are rare in rhupus patients. Our patient diagnosed as rhupus was a young women, presented with multi-organ involvement of systemic vasculitis. Rheumatologists should be aware of possibility that rhupus may be accompanied by progressive or life-threatening conditions such as vasculitis.

scholarly journals Cricoarytenoiditis as an Initial Manifestation of Systemic Lupus Erythematosus

2011 ◽  
Vol 2011 ◽  
pp. 1-3 ◽  
Author(s):  
Jean-Michel Hougardy ◽  
Nicolas Roper ◽  
Alain Michils ◽  
Muhammad S. Soyfoo
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Inflammatory Disease ◽  
Lupus Erythematosus ◽  
Clinical Manifestations ◽  
Chronic Inflammatory Disease ◽  
Vocal Cord Dysfunction ◽  
Systemic Lupus ◽  
Vocal Cords ◽  
Initial Manifestation ◽  
Life Threatening

Systemic lupus erythematosus (SLE) is a chronic inflammatory disease encompassing a broadened spectrum of clinical manifestations. Vocal cords involvement in SLE is not a frequent entity but can be life threatening if not treated. We hereby report the case of a patient presenting with cricoarytenoiditis and vocal cord dysfunction revealing SLE.

Clinical case of lung lesions in patient with newly diagnosed systemic lupus erythematosus

2019 ◽  
Vol 1 (9) ◽  
pp. 53-57
Author(s):  
T. N. Gavva ◽  
L. V. Kuzmenkova ◽  
Yu. N. Fedulaev ◽  
T. V. Pinchuk ◽  
D. D. Kaminer ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Clinical Case ◽  
Clinical Manifestations ◽  
Lung Damage ◽  
Newly Diagnosed ◽  
Systemic Lupus ◽  
Lung Lesions ◽  
Laboratory Parameters ◽  
Clinical Interest

A case of lung damage in systemic lupus erythematosus (SLE) in a 33-year-old woman is described. This case is of clinical interest due to the complexity of diagnosis due to the fact that SLE is a disease with diverse clinical manifestations involving many organs and systems, which often makes it difficult to timely recognize the onset of the disease. SLE still remains a challenge and requires special attention to the patient s history, clinical and laboratory parameters of the patient, as well as specific immunological examinations.

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