Rowell’s syndrome: a rare but distinct entity in rheumatology

2020 ◽  
Vol 13 (9) ◽  
pp. e235173
Author(s):  
Atanu Chandra ◽  
Swarup Kanta Saha ◽  
Aritra Kumar Ray ◽  
Parthasarathi Karmakar
Keyword(s):  
Lupus Erythematosus ◽  
Antinuclear Antibody ◽  
Hard Palate ◽  
Clinical Presentation ◽  
Skin Lesions ◽  
Crust Formation ◽  
Distinct Entity ◽  
Speckled Pattern ◽  
Positive Antinuclear Antibody ◽  
Rowell’S Syndrome

Rowell’s syndrome is a rare disorder characterised by an association of lupus erythematosus with erythema multiforme (EM)-like skin lesions. EM as the initial clinical presentation of systemic lupus erythematosus is also atypical and even rarer. We report the case of an 18-year-old girl admitted to our hospital with fever and polyarthralgia along with multiple discrete ill-defined target lesions with crust formation over forehead, cheek, external ears, scalp, upper chest and back (predominantly over sun-exposed areas) with ulceration over hard palate. Investigations revealed pancytopaenia, a positive rheumatoid factor, positive antinuclear antibody with a speckled pattern, anti-Smith antibody and strongly positive anti-Ro. Patient was diagnosed with Rowell’s syndrome as per clinical and laboratory features. Majority of skin lesions including oral ulcerations subsided gradually after treatment with steroids and hydroxychloroquine.

scholarly journals Association of Immunofluorescence pattern of Antinuclear Antibody with Specific Autoantibodies in the Bangladeshi Population

2015 ◽  
Vol 40 (2) ◽  
pp. 74-78 ◽  
Author(s):  
S Sharmin ◽  
S Ahmed ◽  
A Abu Saleh ◽  
F Rahman ◽  
MR Choudhury ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Lupus Erythematosus ◽  
Antinuclear Antibody ◽  
Research Work ◽  
Connective Tissue Disorder ◽  
Nuclear Antigen ◽  
Dot Blot ◽  
Serum Samples ◽  
Speckled Pattern ◽  
Extractable Nuclear Antigen

Antinuclear antibody (ANA) is useful in the diagnosis of connective tissue disorder (CTD). Association of specific autoantibodies with the immunofluorescence pattern of ANA in CTD, noted in western literature has been considered as reference in all over the world. However, in Bangladesh no such research work or data correlating the autoantibodies and their ANA patterns is found. Objective of the study was to identify an association between immunofluorescence patterns of antinuclear antibody on HEp-2 cell and more specific antinuclear reactivities (e.g. anti-dsDNA and anti-extractable nuclear antigen) in the serum samples of CTD patients. Serum samples of 152 CTD patients (Systemic lupus erythematosus, Rhumatoid arthritis, Sjogren´s syndrome, Systemic sclerosis, Polymyositis, Mixed connective tissue disease) were diagnosed clinically, attending at Bangabandhu Sheikh Mujib Medical University (BSMMU) during the study period of January, 2010 to December, 2010. Samples were subjected for ANA testing by Indirect Immunofluorescence (IIF) on HEp-2 cell (ALPHADIA) in dilution of 1:40, anti-dsDNA by ELISA and anti- extractable nuclear antigen (anti-ENA) by Dot Immunoblot. Dot blot strips were tested for anti-Sm, anti-RNP, anti-SSA/Ro, anti-SSB/La, anti-Scl-70 and anti-Jo-1. Out of 152 patients 110 (72.3%) cases were ANA positive by IIF on HEp-2 cell. ANA positive sera exhibited four fluorescence patterns such as speckled (50.8%), peripheral (21.6%) ,homogenous (18.1%) and nucleolar pattern (9%). Peripheral pattern and homogenous pattern was predominantly associated with anti-dsDNA (p<0.05). Speckled pattern was significantly associated with anti-ENA (p<0.05).The most commonly identified antinuclear autoreactivity was directed towards anti-RNP (25.7%) then anti-Scl-70 (20%), anti-SSA (14.2%) and anti-SSB (5.7%). Multiple anti-ENA reactivities were identified in 34.28% cases. Peripheral and homogenous pattern is strongly associated with anti-dsDNA and speckled pattern may predict anti-ENA (specially ribonucleoprotiens). As a definite correlation between the ANA patterns and the group of antibodies was detected by dot immunoblot, one could predict presence of certain specific auto antibodies for a particular ANA pattern identified. This may restrict on the cost of laboratory investigations in a developing country like Bangladesh. Thus, ANA-IIF method may reduce the expense of detailed immunological work-up with minimal loss in diagnostic accuracy.Bangladesh Med Res Counc Bull 2014; 40 (2): 74-78

scholarly journals Pyoderma Gangrenosum with Positive Antinuclear Antibody, in the Absence of Systemic Association

2018 ◽  
Vol 16 (1) ◽  
pp. 66-69
Author(s):  
Smriti Shrestha ◽  
Alisha Aryal
Keyword(s):  
Pyoderma Gangrenosum ◽  
Lupus Erythematosus ◽  
Antinuclear Antibody ◽  
Case Reports ◽  
Neutrophilic Dermatosis ◽  
Systemic Diseases ◽  
Connective Tissue Disorders ◽  
Systemic Lupus ◽  
Unique Case ◽  
Positive Antinuclear Antibody

Pyoderma gangrenosum is an uncommon neutrophilic dermatosis, seen on legs, and infrequently on hands and other anatomical sites. It is associated with systemic diseases in 50-70% of the cases. Antinuclear antibody (ANA) seropositivity has been reported in pyoderma gangrenosum associated with connective tissue disorders. However, there are very few case reports of pyoderma gangrenosum in patients of systemic lupus erythematosus, while we did not find any reports of ANA seropositivity in isolated pyoderma gangrenosum. Hence, we report this unique case of pyoderma gangrenosum with classical clinicohistopathology, positive ANA but no systemic association. As anticipated, our patient responded promptly to steroids.

Pathogenesis of cutaneous lupus erythematosus: common and different features in distinct subsets

Lupus ◽  
2010 ◽  
Vol 19 (9) ◽  
pp. 1020-1028 ◽  
Author(s):  
J. Wenzel ◽  
S. Zahn ◽  
T. Tüting
Keyword(s):  
Adhesion Molecules ◽  
Complex Network ◽  
Lupus Erythematosus ◽  
Clinical Presentation ◽  
Tissue Injury ◽  
Cutaneous Lupus Erythematosus ◽  
Skin Lesions ◽  
Pathological Features ◽  
Complete Understanding ◽  
Cutaneous Lupus

The term ‘cutaneous lupus erythematosus’ (CLE) comprises several related autoimmune skin disorders, defined as ‘specific’ skin manifestations of lupus erythematosus (LE). The spectrum of clinical presentation of CLE is wide, reaching from mild erythema to disseminated scarring skin lesions. There is increasing knowledge concerning the pathogenesis of LE skin lesions and it has been shown that a complex network of cutaneous cytokines, chemokines and adhesion molecules orchestrate and promote tissue injury observed in LE skin lesions. However, a complete understanding of the diverse pathophysiological mechanisms in the different CLE subsets does not exist. Here we review the main pathological features described in CLE patients against the background of the clinical diversity of different CLE subtypes. Lupus (2010) 19, 1020—1028.

scholarly journals Neutrophil nuclear patterns to distinguish between pathological and non-pathological autoantibodies detected on HEp-2 cells

2020 ◽  
Author(s):  
Dong Il Won ◽  
Jihea Park ◽  
Beom Soo Kim ◽  
Chae Eun Kim ◽  
Heon Sik Yoon ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Antinuclear Antibody ◽  
Cell Nuclei ◽  
Systemic Lupus ◽  
Speckled Pattern ◽  
Autoimmune Rheumatic Disease ◽  
Diffuse Systemic Sclerosis ◽  
Systemic Autoimmune Rheumatic Disease

AbstractBackgroundAntinuclear antibody (ANA) testing is used to diagnose systemic autoimmune rheumatic disease (SARD). Autoantibodies (Abs) associated with the “homogeneous-like” pattern on ANA HEp-2 cell nuclei can be classified as pathological (e.g., anti-dsDNA, anti-nucleosome, anti-histone, anti-Scl-70 Abs) or non-pathological (e.g., anti-DFS70 Abs).MethodsAnti-neutrophil cytoplasmic anti-antibody (ANCA) testing was used to classify individuals who presented with a homogeneous-like pattern on ANA testing. Enrolled subjects included (1) young individuals with a dense fine speckled pattern on ANA testing (young non-SARD group, n = 62) and patients with (2) systemic lupus erythematosus (SLE) with anti-dsDNA Abs (SLE group, n = 33), (3) rheumatoid arthritis (RA) with anti-nucleosome, anti-histone Abs, and others (RA group, n = 45), and (4) diffuse systemic sclerosis (SSc) with Scl-70 Abs (diffuse SSc group, n = 11).ResultsNegative rates (95% confidence interval) of neutrophil nuclear patterns on ANCA testing were: 96.8% (88.8%–99.6%) of the young non-SARD group, 3.0% (0.1 %–15.8%) of the SLE group, 4.4% (0.5%–15.2%) of the RA group, and 54.5% (23.4%–83.3%) of the diffuse SSc group. The negative rate of the non-SARD group was significantly higher than those of the SARD group (all P < 0.05).ConclusionsANCA testing helps to identify individuals with non-pathological anti-DFS70 Abs who present with homogeneous-like patterns in HEp-2 cell nuclei on ANA testing.

scholarly journals Immunofluorescence pattern of antinuclear antibody and its association with autoantibody profile in systemic lupus erythematosus

2016 ◽  
Vol 6 (2) ◽  
pp. 141
Author(s):  
Sadia Sharmin ◽  
Sharmeen Ahmed ◽  
Humayun Sattar ◽  
Md. Ruhul Amin Miah ◽  
Minhaz Rahim Chowdhury ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Antinuclear Antibody ◽  
Research Work ◽  
Nuclear Antigen ◽  
Dot Blot ◽  
Systemic Lupus ◽  
Serum Samples ◽  
Speckled Pattern ◽  
Extractable Nuclear Antigen

<p><strong>Background:</strong> Antinuclear antibody (ANA) is useful in the diagnosis of systemic lupus erythematosus (SLE). Association of specific autoantibodies with the immunofluorescence pattern of ANA in SLE as noted in Western literature has been taken as reference in all over the world. However, in Bangladesh such research work or data correlating the autoantibodies and their ANA patterns is inadequate. <strong></strong></p><p><strong>Objective:</strong> To identify an association between immunofluorescence patterns of antinuclear antibody on HEp-2 cell and more specific antinuclear reactivities (e.g. anti-dsDNA and anti-extractable nuclear antigen) in the serum samples of SLE patients.</p><p><strong>Methods:</strong> Serum samples of 37 SLE patients who were diagnosed by ARA (American Rheumatism Association) classification criteria and laboratory tests, attending at lupus clinic of Bangabandhu Sheikh Mujib Medical University (BSMMU) during the study period of six months were subjected for ANA testing by Indirect Imrnunofluorescence (IIF) on HEp-2 cell, anti-dsDNA by ELISA and anti- extractable nuclear antigen (anti-ENA) by Dot Immunoblot. Dot blot strips were tested for anti-Sm, anti-RNP, anti-SSA/Ro, and anti-SSB/La. <strong></strong></p><p><strong>Results:</strong> Out of 37 SLE patients 32 (86.5%) cases were ANA positive by IIF on HEp-2 cell. ANA positive sera exhibited three fluorescence patterns such as speckled (43.7%), peripheral (34.3%) and homogenous pattern (21.8%). Peripheral pattern (100%) was strongly associated with anti-dsDNA (p&lt;0.05) and homogenous pattern (85.7%) was also predominantly associated with anti-dsDNA (p&lt;0.05). Speckled pattern (85.6%) was significantly associated with anti-ENA (p&lt;0.05). Anti-dsDNA was positive in 75% of SLE cases and majority (45.8%) of which showed peripheral pattern whereas anti-ENA was positive in 48.6% cases and majority (70.5%) of which showed speckled pattern. The most commonly identified antinuclear autoreactivity was directed towards anti-RNP (22.2%) then anti-Sm (16.6%), anti-SSA (16.6%) and anti-SSB (11.1 %). Multiple anti-ENA reactivities were identified in 33.3% cases. <strong></strong></p><p><strong>Conclusion:</strong> Peripheral and homogenous pattern is strongly associated with anti-dsDNA therefore may be predicted that patients have active SLE and speckled pattern may predict anti-ENA (specially ribonucleoprotiens). Thus, ANA-IIF method may suffice and probably reduce the expense of detailed immunological work-up with minimal loss in diagnostic accuracy.</p>

scholarly journals THU0284 PREVALENCE OF NEUROPSYCHIATRIC LUPUS IN PSYCHOSIS PATIENTS WITH A POSITIVE ANTINUCLEAR ANTIBODY

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 369.1-369
Author(s):  
M Spies ◽  
J Gutjahr-Holland ◽  
J. V. Bertouch ◽  
A Sammel
Keyword(s):  
Mental Health ◽  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Antinuclear Antibody ◽  
Systemic Lupus ◽  
Positive Antinuclear Antibody ◽  
Rare Manifestation

Background:Psychosis is a rare manifestation of Neuropsychiatric Systemic Lupus Erythematosus (NPSLE). Patients with SLE may have Psychosis as part of their initial presentation of disease1. Current guidelines do not make a recommendation regarding the use of Antinuclear Antibody (ANA) in the assessment of patients with psychosis2. There is limited evidence assessing the utility of ANA testing in this setting.Objectives:Primary objective: Determine the prevalence of NPSLE in patients admitted to a mental health service with a diagnosis of a psychosis, who have had a positive antinuclear antibody test.Secondary objectives: Determine the frequency and proportion of positive ANA testing in this patient group. Determine the pattern and titres of positive ANAs. Determine the subsequent investigation, referral and diagnosis of patients with positive ANAs.Methods:Retrospective chart review of patients admitted to a mental health service of two metropolitan tertiary referral centres, Prince of Wales Hospital (POWH) and Royal Prince Alfred Hospital (RPAH), with a diagnosis of psychosis who had been tested for ANA. Patients were identified using their electronically entered diagnosis based on the International Classification of Disease3codes. Assessment of patient data for SLE used the 2019 ACR/EULAR classification criteria4. Decisions regarding attribution of psychosis related events to SLE follows the criteria used by Bortoluzzi et al5.Results:Between 1stof January 2010 and 31stof March 2018 there were 5585 (POWH) and 4620 (RPAH) mental health admission with an ICD diagnosis of psychosis representing 2451 and 2315 individual patients. 449/2451 (18%) and 462/2315 (20%) patients were tested for ANA. 78/449 (17%) and 57/462 (12%) were positive. Discharge data was available for all patients and long-term follow up data was completed for 53/78 (81% - POWH) patients and 50/57 (88% - RPAH). The mean follow-up time 43 ± 23 months and 51 ± 29 months respectively.At discharge there were four patients who met 2019 ACR/EULAR for SLE. Of these, two patients met criteria for NPSLE. One was diagnosed clinically and treated specifically for NPSLE with intravenous methylprednisolone and rituximab.There were no additional diagnoses of SLE or NPSLE clinically or by criteria found in the available follow up data. Hence the overall prevalence of NPSLE in patients admitted with psychosis was 1.3%, 95%CI [0,6.9%] and 1.8%, 95%CI [0,9.4%] respectively.Conclusion:The prevalence of neuropsychiatric lupus in patients with psychosis and a positive ANA was 1/78 and 1/57 a two tertiary referral centres. This study expands significantly on the limited evidence available as to the expected outcomes of a positive ANA test in a patient with psychosis.References:[1]Pego-Reigosa JM, Isenberg DA. Psychosis due to systemic lupus erythematosus: characteristics and long-term outcome of this rare manifestation of the disease. Rheumatology (Oxford). 2008; 47:1498–502.[2]National Institute for Health and Care Excellence. Psychosis and schizophrenia in adults: prevention and management. London: NICE; 2014. Clinical guideline CG178.[3]World Health Organization. (2004). ICD-10: international statistical classification of diseases and related health problems: tenth revision, 2nd ed.[4]Aringer M, et al. 2019 European League Against Rheumatism/American College of Rheumatology classification criteria for systemic lupus erythematosus. Arthritis Rheum. 2019; 71(9): 1400–1412.[5]Bortoluzzi A, et al. Development and validation of a new algorithm for attribution of neuropsychiatric events in systemic lupus erythematosus. Rheumatology. 2015; 54: 891-898.Acknowledgments:Gordana Popovic, Statistical Consultant, Stats Central, Mark Wainwright Analytical Centre, University of New South Wales, Sydney, Australia.Disclosure of Interests:None declared

scholarly journals Spontaneous soft tissue haemorrhage in systemic lupus erythematosus

Reumatismo ◽  
2016 ◽  
Vol 68 (4) ◽  
pp. 199 ◽  
Author(s):  
M.C. Abdulla
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Soft Tissue ◽  
Lupus Erythematosus ◽  
Antinuclear Antibody ◽  
Low Grade ◽  
Systemic Lupus ◽  
Double Stranded Dna ◽  
Positive Antinuclear Antibody ◽  
Clinical Presentations ◽  
Retinal Haemorrhages

Diversity in clinical presentations and complications of systemic lupus erythematosus (SLE) make the diagnosis and management challenging. The mechanisms of haemorrhagic manifestations in SLE have not been well elucidated. A 47-year-old woman with no comorbidities was admitted after suffering fatigue and low grade fever for six months. She had bilateral soft tissue haemorrhage over the forearm and intra retinal haemorrhages. She was assessed and diagnosed as having SLE based on positive antinuclear antibody, strongly positive anti double stranded DNA, thrombocytopenia and low C3 and C4 levels. We describe a case of spontaneous bilateral soft tissue haemorrhage in SLE and discuss the various mechanisms causing bleeding in lupus.

Generalized Canine Discoid Lupus Erythematosus Responsive to Tetracycline and Niacinamide Therapy

2015 ◽  
Vol 51 (3) ◽  
pp. 171-175 ◽  
Author(s):  
Michael A. Rossi ◽  
Linda M. Messinger ◽  
Keith E. Linder ◽  
Thierry Olivry
Keyword(s):  
Complete Remission ◽  
Lupus Erythematosus ◽  
Clinical Presentation ◽  
Clinical Signs ◽  
Skin Lesions ◽  
Direct Immunofluorescence ◽  
Discoid Lupus Erythematosus ◽  
Topical Tacrolimus ◽  
Systemic Glucocorticoids ◽  
Atrophic Scars

Discoid lupus erythematosus (DLE) is a commonly reported canine autoimmune disease that normally presents with a phenotype consisting of erythema, depigmentation, scaling, erosions/ulcers, and scarring over the nasal planum and the proximal dorsal muzzle. Recently, two cases of a generalized variant of this disease have been reported, whose lesions responded to either systemic glucocorticoids or a combination of topical corticosteroids, topical tacrolimus, and the oral antimalarial hydroxychloroquine. The purpose of this report is to describe an 11 yr old shih tzu that presented with skin lesions consisting of multiple annular, erythematous papules and plaques, hyperpigmentation, adherent scaling, and atrophic scars over the caudal dorsum, flanks, craniodorsal thorax, and lateroproximal extremities. A diagnosis of generalized DLE was made based on the clinical presentation, histopathology, laboratory values, and direct immunofluorescence findings. Treatment consisted of oral tetracycline and oral niacinamide, which resulted in complete remission of clinical signs. This is the first documented report of generalized canine DLE responding to the described immunomodulating regimen. Such a combination might therefore be considered as a glucocorticoid and/or antimalarial alternative for the management of generalized DLE.

scholarly journals The Protective Effects of Ultraviolet A1 Irradiation on Spontaneous Lupus Erythematosus-Like Skin Lesions in MRL/lpr Mice

2009 ◽  
Vol 2009 ◽  
pp. 1-9 ◽  
Author(s):  
Naoya Mikita ◽  
Nobuo Kanazawa ◽  
Takashi Yoshimasu ◽  
Takaharu Ikeda ◽  
Hong-jin Li ◽  
...  
Keyword(s):  
Mast Cells ◽  
Disease Prevention ◽  
Lupus Erythematosus ◽  
Antinuclear Antibody ◽  
Skin Lesions ◽  
Apoptotic Cells ◽  
Protective Effects ◽  
Prevention Model ◽  
Antibody Levels ◽  
Uva1 Irradiation

We investigated the effects of ultraviolet A1 (UVA1) irradiation on spontaneous lupus erythematosus- (LE-) like skin lesions of MRL/lpr mice, using a disease prevention model. UVA1 irradiation significantly inhibited the development of LE-like skin lesions, without obvious changes of the disease including renal disease and serum antinuclear antibody levels. Besides the massive infiltration of mast cells in the LE-like skin lesions, in the nonlesional skins, more mast cells infiltrated in the UVA1-irradiated group compared with the nonirradiated group. Although apoptotic cells were remarkably seen in the dermis of UVA1-irradiated mice, those cells were hardly detectable in the dermis of the nonirradiated mice without skin lesions. Further analysis showed that some of those apoptotic cells were mast cells. Thus, UVA1 might exert its effects, at least in part, through the induction of the apoptosis of pathogenic mast cells. Our results supported the clinical efficacy of UVA1 irradiation for skin lesions of lupus patients.

Sign in / Sign up

Export Citation Format

Share Document