scholarly journals Rowell syndrome in dermatological practice (a clinical case)

2019 ◽  
Vol 47 (5) ◽  
pp. 470-476
Author(s):  
A. V. Taganov ◽  
O. B. Tamrazova ◽  
M. A. Gureeva
Keyword(s):  
Rheumatoid Factor ◽  
Lupus Erythematosus ◽  
Clinical Case ◽  
Clinical Signs ◽  
Clinical Manifestations ◽  
Direct Immunofluorescence ◽  
Positive Rheumatoid Factor ◽  
Skin Symptoms ◽  
Incorrect Diagnosis ◽  
Methylprednisolone Aceponate

Rowell syndrome is a  rare cluster of symptoms characterized by clinical manifestation of lupus erythematosus and erythema multiforme (EM). About 100  cases of the syndrome have been reported in medical publications during the last 100 years. This may be related to misinterpretation of the symptoms and subsequent incorrect diagnosis due to its EM-like manifestations. Important clues for the diagnosis of Rowell syndrome are findings of positive rheumatoid factor, anti-nuclear antibodies and other erythematoid markers, as well as additional investigations, in particular, direct immunofluorescence technique. The paper describes a  clinical case of Rowell syndrome in a 16-year old male patient. The diagnosis was challenging due to EM-like skin manifestations and required additional laboratory work-up, as well as the patient's follow-up. The diagnosis of Rowell syndrome was based on the clinical manifestations and on such diagnostic criteria as positive rheumatoid factor and anti-nuclear antibodies, as well as histological and laboratory abnormalities characteristic of the erythematosis. The patient was hospitalized and received the following treatment: prednisolone infusion (2.5 mg/kg/daily for 7 days), chloropyramine (1 mL i.m. twice daily for 5  days), hydroxychloroquine (6.5  mg/kg daily for 5  days), magnesium asparaginate/potassium asparaginate (one tablet (166.3  mg/175  mg) 3  times daily for 7  days), topical methylprednisolone aceponate cream 1% (once daily for 7 days). The treatment resulted in positive changes in the skin lesion and improvement of his general state. This clinical observation gives an example of classic Rowell syndrome proven both by lab and clinical signs, taking into account skin symptoms of lupus erythematosus and EM-like rash.

The lupus patient with positive rheumatoid factor

Lupus ◽  
2018 ◽  
Vol 27 (8) ◽  
pp. 1368-1373 ◽  
Author(s):  
A Fedrigo ◽  
T A F G dos Santos ◽  
R Nisihara ◽  
T Skare
Keyword(s):  
Rheumatoid Factor ◽  
Lupus Erythematosus ◽  
Univariate Analysis ◽  
Disease Onset ◽  
Clinical Manifestations ◽  
Epidemiological Data ◽  
Positive Rheumatoid Factor ◽  
Serological Profile ◽  
Oral Ulcers ◽  
Rheumatology Unit

Background Patients with systemic lupus erythematosus (SLE) may form clusters with clinical manifestations and autoantibodies. Objective The objective of this report is to study whether SLE patients with positive rheumatoid factor (RF) have a special clinical and/or serological profile. Methods A retrospective study of 467 SLE patients seen at a single rheumatology unit was conducted. Epidemiological data (age, gender, age at disease onset, ethnic background and tobacco use), clinical data (malar rash, photosensitivity, oral ulcers, discoid lesions, serositis, glomerulonephritis, convulsions, psychosis, hemolytic anemia, leukopenia, lymphocytopenia, arthritis and hypothyroidism) and serological profile (anti-dsDNA, anti-Ro/SS-A, anti-La/SS-B, anti-RNP, anti-Sm, IgG aCL, IgM aCL, lupus anticoagulant, direct Coombs and RF) were collected. Patients with positive and negative RF were compared. Results RF was found in 24.9% of the sample. In univariate analysis, RF was positively associated with butterfly rash ( p = 0.04), anti-Ro ( p = 0.03), anti-Sm antibodies ( p = 0.01) and hypothyroidism ( p = 0.01) and negatively associated with glomerulonephritis ( p = 0.003). Logistic regression showed that only glomerulonephritis ( p = 0.03; OR = 0.45; 95% CI = 0.21–0.93) and anti-Ro ( p = 0.009; OR = 2.3; 95% CI = 1.24–4.57) were independent associations. Conclusion In our sample RF was associated with protection from glomerulonephritis and with higher prevalence of anti-Ro antibodies.

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.

scholarly journals Predictors of mortality in children with lupus nephritis

2014 ◽  
Vol 54 (6) ◽  
pp. 338
Author(s):  
Lukman Oktadianto ◽  
Risky Vitria Prasetyo ◽  
Ninik Asmaningsih Soemyarso ◽  
Mohammad Sjaifullah Noer
Keyword(s):  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Renal Involvement ◽  
Hypertensive Crisis ◽  
Clinical Signs ◽  
Clinical Manifestations ◽  
Predictors Of Mortality ◽  
Cox Proportional Hazard ◽  
Kaplan Meier ◽  
Mean Time

Background Renal involvement during the clinical course ofsystemic lupus erythematosus (SLE) is generally considered to bethe most important factor influencing disease prognosis in termsof morbidity and mortality. Various factors have been reported toinfluence the prognosis of lupus nephritis (LN).Objective To analyze clinical signs and laboratory parameters thatmight serve as predictors associated with mortality in pediatricLN.Methods Retrospectively, medical records of children with LNat Soetomo Hospital from 1998 to 2011 were studied. Diagnosisof SLE was based on Revised American Rheumatism Associationcritera, while patients with clinical manifestations of hypertension,abnormal urinalysis, and serum creatinin > 1 mg/dL wereconsidered as lupus nephritis. Cox proportional hazard modelingwas used to assess for associations of clinical signs and laboratoryparameters with mortality. Kaplan-Meier survival analysis wasused to assess the cumulative survival from the time of diagnosisto the outcome.Results There were 57 children with LN of whom 43 (75%) weregirls. The female-to-male ratio was 3:1. Subjects’ mean age was 10.6(SD 6.87) years. The mean time of observation was 51 (SD 74.54)months and 23 (40%) children died. Age, gender, hypertension,hematuria, proteinuria, and anemia were not significant aspredictors for mortality. However, hypertensive crisis (HR=2.79;95%CI 1.16 to 6.75; P=0.02) and initial glomerular filtration rate(GFR) of <75 mL/min/1.73m2 (HR=3.01; 95%CI 1.23 to 7.34;P=0.01) were significant predictors of mortality in children with LN.The mean survival time of LN with hypertensive crisis and initialGFR <75 mL/min/1.73m2 was 36.9 (SD 12.17) months.Conclusion Hypertensive crisis and GFR <75 mL/min/1.73m2 aresignificant predictors of mortality in children with LN.

scholarly journals Evolution of Mixed Connective Tissue Disease in 7 Years Old Child: Clinical Case

2020 ◽  
Vol 19 (3) ◽  
pp. 214-219
Author(s):  
Tamara P. Makarova ◽  
Khakim M. Vakhitov ◽  
Dina R. Sabirova ◽  
Dinara I. Sadykova ◽  
Liliya R. Khusnutdinova ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Lupus Erythematosus ◽  
Mixed Connective Tissue Disease ◽  
Clinical Case ◽  
Clinical Signs ◽  
Systemic Scleroderma ◽  
Systemic Diseases ◽  
Autoimmune Pathology ◽  
Short Period

Background. Mixed connective tissue disease (Sharp syndrome) is the rare chronic autoimmune pathology combining various features of systemic lupus erythematosus, systemic scleroderma, rheumatoid arthritis, dermatomyositis and high antibody titer to nuclear ribonucleoprotein. The mixed connective tissue disease may evolve into other systemic diseases over time. Description of any cases of mixed connective tissue disease and its evolution in Russian patients has not been published previously.Clinical Case Description. The results of observations of the child with clinical and immunological signs of the mixed connective tissue disease followed by the progression of systemic scleroderma symptoms and development of Sjogren's syndrome in the short period of time are presented in the article. Improvement (such as pain attenuation, increase in volume of movements in affected joints, decrease of Raynaud syndrome manifestations duration) was observed on treatment (methotrexate 10 mg/week with subsequent addition of prednisolone 0.75 mg/kg/day).Conclusion. Timely diagnostics of clinical signs of the systemic diseases debut is crucial for correct patient routing and for achieving of disease improvement.

scholarly journals Lupus Erythematosus: Cutaneous Manifestations and Treatment

2019 ◽  
Vol 77 (3) ◽  
pp. 201-214
Author(s):  
Francisca Alves ◽  
Margarida Gonçalo
Keyword(s):  
Lupus Erythematosus ◽  
Systemic Disease ◽  
Cutaneous Lupus Erythematosus ◽  
Disease Patient ◽  
Clinical Manifestations ◽  
Disease Diagnosis ◽  
Direct Immunofluorescence ◽  
Discoid Lupus Erythematosus ◽  
Topical Steroids ◽  
Cutaneous Manifestations

Cutaneous lupus erythematosus (CLE) includes a broad range of dermatologic manifestations, which may or may not be associated with systemic manifestations. Specific CLE - defined by the presence of an interface dermatitis on histopathological evaluation - is divided into several sub-types, namely acute CLE (ACLE), subacute CLE (SCLE) and chronic CLE (CCLE). CCLE includes discoid lupus erythematosus (DLE), as well as other rarer forms such as LE profundus (LEP). Nonspecific skin findings, such as livedo reticularis or purpura are more frequently seen in patients with systemic disease. Diagnosis requires classification of the subtype, through a combination of physical examination, laboratory studies, histology and sometimes direct immunofluorescence, at the same time ensuring to exclude systemic disease. Regarding the treatment of CLE, antimalarials and topical steroids continue to be the standard of care; however, immunosuppressants, thalidomide analogs and monoclonal antibodies are possible systemic therapies for recalcitrant disease. Patient education on proper sun protection and avoidance of triggers is crucial. This paper reviews the clinical manifestations of CLE, as well as the treatment.

scholarly journals Challenges of the differential diagnosis between the subtypes of the junctional epidermolysis bullosa: presentation of two clinical cases

2019 ◽  
Vol 47 (1) ◽  
pp. 83-93
Author(s):  
Yu. Yu. Kotalevskaya ◽  
N. M. Marycheva
Keyword(s):  
Differential Diagnosis ◽  
Epidermolysis Bullosa ◽  
Skin Disease ◽  
Clinical Case ◽  
Molecular Genetic ◽  
Clinical Signs ◽  
Clinical Manifestations ◽  
Junctional Epidermolysis Bullosa ◽  
Reliable Determination ◽  
Clinical Cases

Background: Epidermolysis bullosa (EB) is a rare hereditary skin disease. It is subdivided into EB simplex (EBS), junctional EB (JEB), dystrophic EB (DEB) and Kindler syndrome. JEB is diagnosed in 2 per 1,000,000 of the population. There are few descriptions of clinical JEB cases in the literature. Clinical diagnosis of JEB and its subtypes is a challenge, especially in the early age. The paper presents 2 clinical cases of JEB in patients of the West Slavonic origin. Clinical case No. 1 was a girl of Ukrainian ethnicity, with confirmed definitive diagnosis of severe generalized JEB. Molecular genetic tests identified mutations of the LAMA3 gene that had not been described previously. The patient died at the age of 24 months from acute respiratory failure. When the patient was alive, her EB type and subtype was not possible to identify, because she had a combination of clinical manifestations typical for various JEB subtypes. Despite such symptoms as hoarse voice, stenoses, granulation tissue of typical location, laryngeal granulations, the girl was steadily gaining weight, with some periods of relative stabilization of the skin disease; she also had longer life longevity than was common for patients with severe generalized JEB. All this made a precise diagnosis difficult. Clinical case No. 2: an ethnic Russian boy with non-classified JEB. Molecular genetic testing helped to identify a homozygote mutation in the LAMA3 gene that had not been previously described; reliable determination of the subtype was not possible. The patient had mixed clinical manifestation similar both to generalized severe JEB and to laryngo-onycho-cutaneous (LOC) syndrome. During his lifetime, the patient was clinically diagnosed with Hallopeau acrodermatitis and LOC syndrome. The differential diagnostic problems were associated with the presence of signs not typical for each of the subtypes. Significant life longevity of the proband is not characteristic for severe generalized JEB (at the time of the publication the patient is 13 years old), whereas for LOC syndrome the absence of eye involvement is not typical, as well as severe laryngeal involvement at adolescence.Conclusion: Detailed descriptions of phenotype of JEB subtypes including rare and minimal clinical signs can be useful to study the clinical manifestations and natural course of the disease, including its differential diagnosis.

Development of sulfasalazine-associated drug-induced lupus erythematosus in patient with rheumatoid arthritis (clinical case)

2021 ◽  
pp. 26-29
Author(s):  
A. N. Kovshik ◽  
E. P. Kiseleva ◽  
N. G. Klyukvina ◽  
G. V. Lukina
Keyword(s):  
Rheumatoid Arthritis ◽  
Lupus Erythematosus ◽  
Clinical Case ◽  
Clinical Manifestations ◽  
New Drugs ◽  
Drug Induced ◽  
Reliable Diagnosis ◽  
Term Administration ◽  
Lupus Syndrome

Drug-induced lupus syndrome (DLS) is a rare adverse event with a variety of drugs. More than a hundred of drugs are known that can cause the development of DLS, and this list is growing as new drugs appear. Physicians of any specialty can face such complications of therapy and should be aware of this pathology. The article presents an analysis of a clinical case of DLS development against the background of long-term administration of sulfasalazine in a patient with a reliable diagnosis of rheumatoid arthritis, as well as a literature review, which includes data on the prevalence, drug groups, clinical manifestations, diagnosis and treatment of this pathology.

SYSTEMIC LUPUS ERYTHEMATOSUS: REVIEW OF MODERN METHODS OF DIAGNOSIS, TREATMENT AND DESCRIPTION OF THE CLINICAL CASE

2021 ◽  
pp. 57-65
Author(s):  
Nadezhda V. Zhuravleva ◽  
Louise M. Karzakova ◽  
Tatyana L. Smirnova ◽  
Sergey I. Kudryashov ◽  
Tatyana S. Lutkova ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Rheumatoid Factor ◽  
Lupus Erythematosus ◽  
Clinical Case ◽  
Skin Lesions ◽  
Blood Analysis ◽  
Federal State ◽  
Systemic Lupus ◽  
Initial Examination ◽  
Immunological Disorders

A clinical case of systemic lupus erythematosus (SLE) is presented. Since childhood the patient had preclinical lupus: leukopenia, anemia, photoallergy, vasculitis, and serological changes a-SS-A (+++), but at that time the findings did not meet the criteria for SLE. At the initial examination of the woman at the age of 24 years, a clinical blood test revealed hemoglobin 101 g/l, leukopenia 3.2×109/l, ESR 41 mm/h, hyper-γ-globulinemia 29%. The immunological blood analysis revealed a-DNA (+++), a-SS-A (+++), aSm (+++), aRNP (+++). The diagnosis was "Systemic lupus erythematosus of chronic (at the beginning) course, activity of the 1st degree (2 points by the SLEDA scale): with hematological disorders (leukopenia), immunological disorders (ANA+)". Metypred was administered at a dose of 16 mg/day, followed by the dose reduction to 6 mg/day and Plaquenil 400 mg/day. The woman received inpatient and outpatient treatment, repeatedly consulted in the Federal State Budgetary Institution "V.A. Nasonova Research Institute" under the Russian Academy of Medical Sciences. At the age of 26, the diagnosis was made: "Systemic lupus erythematosus, grade 2 activity with skin lesions of the type of subacute cutaneous lupus, cheilitis, lymphadenopathy", blood tests revealed leukopenia, lymphopenia, anemia, as well as immunological disorders: a-n DNA 93.4 u/ml, aSm > 200 u/ml, aRo > 200 u/ml, C3 0.63 g/l, rheumatoid factor 69.5 mIU/ml, ANA (Нер-2) 1/640 Sp. A complex therapy was performed with the use of Metypred, Cyclophosphane and Azathioprine. Against the background of the therapy, the patient's condition is stable, but immunological disorders are preserved: persistent high positivity for aRo-SS-A and the presence of the rheumatoid factor. The case demonstrates the need for an in-depth examination of women with anemia of unknown origin in combination with leukopenia to exclude systemic diseases of the connective tissue.

scholarly journals SYNDROME OF THE FIRST GILL ARCH IN A NEWBORN CHILD (CLINICAL CASE)

2020 ◽  
Vol 20 (4) ◽  
pp. 237-240
Author(s):  
M. Yе. Fesenko ◽  
O. A. Scherban ◽  
M. M. Fastovets ◽  
O.O. Kalyuzhka ◽  
Yu. I. Chernyavska
Keyword(s):  
At Risk ◽  
Clinical Case ◽  
Clinical Signs ◽  
Clinical Manifestations ◽  
Facial Asymmetry ◽  
Final Diagnosis ◽  
Gill Arch ◽  
Dynamic Monitoring ◽  
Large Variability ◽  
Neuropsychological Development

The article describes a clinical case of “First Gill Arch Syndrome" in a newborn girl, the peculiarities of the syndrome, its diagnosis. The characteristics of this disease is that the mother of the child was at risk group due to smoking, anaemia during the pregnancy and medical abortions in the past obstetric history. The aetiology of "First Gill Arch Syndrome" is insufficiently studied, but, according to latest concepts, this condition results from the mutations in the TCOF1 gene. The aetiology of the disease also does not exclude the role of adverse obstetric and gynaecological anamnesis and diseases of the mother, previous medical abortions and teratogenic factors. Difficulties in diagnosis are due to the large variability of clinical manifestations and course of the disease. The final diagnosis of the child was based on specific clinical signs of the disease: facial asymmetry, unilateral facial paralysis (lesions of the facial nerve on the right), the presence of blind fistula of the left cheek and skin suspension of the left auricle. We can conclude about the necessity to elaborate preventive measures to reduce the occurrence of this disease: timely ultrasound examination of pregnant women, who are at risk and mandatory dynamic monitoring of a child with this disease to assess physical and neuropsychological development.

scholarly journals Increased CD21-CD27- atypical memory B cells as a biomarker of clinical disease activity in patients with systemic lupus erythematosus

2021 ◽  
Author(s):  
Alice Horisberger ◽  
Morgane Humbel ◽  
Natalia Fluder ◽  
Florence Bellanger ◽  
Craig Fenwick ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
B Cells ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Clinical Signs ◽  
Clinical Manifestations ◽  
Memory B Cells ◽  
Clinical Disease ◽  
Systemic Lupus ◽  
Clinical Disease Activity

Abstract Background Determining disease activity in systemic lupus erythematosus (SLE) patients is challenging and limited by the lack of reliable biomarkers. Analysis of the altered distribution of circulating B cells has shown promise for assessment disease activity. Yet studies are limited by variable classifications of B subsets. Therefore, we studied peripheral B cells using high-dimensional tools to identify a valid biomarker of disease activity.Methods We studied B cells in two separate cohorts of patients included in the Swiss SLE Cohort Study. In discovery cohort A, we analyzed cryopreserved PBMCs from 30 SLE, and 30 age-, sex- and ethnicity matched healthy controls (HC) by mass cytometry. In validation cohort B, fresh blood from 63 SLE, 14 Sjögren syndrome (pSS), 14 Sarcoidosis (Sarc), and 39 age-matched HC were analyzed by flow cytometry.Results In cohort A, using unsupervised clustering analysis, we identified 7 metaclusters within B cells. Two metaclusters were increased in SLE and exhibited a phenotype of atypical memory B cells (aMBC): CD21-, CD27-, CD11c+ and CXCR5-. Based on cohort A results, we confirmed in cohort B the increase in CD21-CD27- aMBC in SLE, compared to healthy and disease controls. In both cohorts, aMBC were associated with the severity of clinical manifestations. Compared to classical biomarkers, aMBC showed a significant correlation with clinical signs of disease activity. Conclusion aMBC were expanded in SLE, and the increase correlated with clinical disease activity. According to our data, aMBC represents a robust and easily accessible biomarker to assess disease activity in patients with SLE.

Sign in / Sign up

Export Citation Format

Share Document