scholarly journals Lupus Never Fails to Deceive US: A Case of Rowell’s Syndrome

2020 ◽  
Vol 2020 ◽  
pp. 1-4
Author(s):  
Ana B. Arevalo ◽  
Rawann Nassar ◽  
Satyam Krishan ◽  
Priyanka Lakshmanan ◽  
Maria Salgado ◽  
...  

Background. Rowell’s syndrome is comprised of the presentation of erythema multiforme- (EM-) like lesions in association with lupus erythematosus (LE), along with serologies of speckled antinuclear antibodies (ANAs), positive rheumatoid factor (RF), positive anti-La/anti-Ro, and the clinical finding of chilblains. As per the redefined criteria by Zeitouni et al., three major criteria in addition to at least 1 minor criterion are necessary for diagnosis. Case Presentation. A 20-year-old male presented with a one-week history of worsening nonpruritic erythematous maculopapular skin rash (resembling EM) which appeared on the face and subsequently spread to the trunk, arms, legs, palms, and soles. There was no mucosal involvement. At the onset of rash, the patient reported headaches, associated with photosensitivity and intermittent fevers. Workup for viral meningitis yielded negative results. Laboratory investigation revealed mild anemia, elevated inflammatory markers, a positive ANA with speckled pattern, a positive anti-Ro/SSA, anti-La/SSB antibodies, and a positive rheumatoid factor (RF). Lupus anticoagulant antibody was positive along with a low-positive anticardiolipin IgM antibody and a negative beta-2 glycoprotein antibody. Anti-dsDNA, anti-Smith, anti-Jo-1, anti-centromere, and anti-Scl-70 antibodies were negative. Hepatitis serologies, herpes simplex virus 1 and 2, mycoplasma, Epstein–Barr virus, HIV, and parvovirus B19 were negative. Left arm skin biopsy demonstrated vacuolar interface dermatitis and positive colloidal iron stain suggestive of dermal mucin deposition, favoring the diagnosis of cutaneous collagen vascular disease. Cutaneous lesions improved with administration of oral prednisolone. Conclusion. Rowell’s syndrome should be considered in patients who present with cutaneous LE and lesions resembling EM. Further serological markers should be pursued in the absence of obvious EM-precipitating factors.

Lupus ◽  
2018 ◽  
Vol 27 (8) ◽  
pp. 1368-1373 ◽  
Author(s):  
A Fedrigo ◽  
T A F G dos Santos ◽  
R Nisihara ◽  
T Skare

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.


2019 ◽  
Vol 47 (5) ◽  
pp. 470-476
Author(s):  
A. V. Taganov ◽  
O. B. Tamrazova ◽  
M. A. Gureeva

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.


2021 ◽  
Author(s):  
Elham Beshir ◽  
Ernestina Belt ◽  
Nidheesh Chencheri ◽  
Aqdas Ismail ◽  
Marco Pallavidino ◽  
...  

Abstract Background: Peripheral nervous system (PNS) involvement, including Guillain Barré Syndrome (GBS), accounts for fewer than 10% of SLE cases with neuropsychiatric manifestations. GBS as the presenting, major manifestation of pediatric SLE is extremely rare, and the best treatment approach is unknown. Case presentation: A 14-year-old, previously healthy female Emirati teenager presented with a classic picture of GBS with ascending, progressive bilateral muscle weakness leading to respiratory insufficiency within five weeks of symptom onset, associated with typical protein-cell dissociation in cerebro-spinal fluid and nerve root enhancement demonstrated by spinal MRI. Subsequently, elevated anti-dsDNA and anti-Smith/RNP and anti-SS-A and -B antibody concentrations were detected in serum, suggestive of SLE. GBS treatment was initiated with IVIG and methylprednisolone pulses, with minimal improvement. The patient required endotracheal intubation and ventilation, followed by a second course of IVIG, rituximab, and eventually plasma exchange (PLEX) therapy. The diagnosis of lupus-associated GBS was corroborated by a kidney biopsy demonstrating lupus nephritis WHO class II with “full house” immunofluorescence pattern. After 14 PLEX sessions, her muscle strength and respiratory efforts had improved substantially. Treatment was completed with two more rituximab infusions, followed by mycophenolate mofetil, in addition to HCQ and tapering doses of oral prednisolone. Five weeks after the last PLEX treatment, she had regained her usual strength and achieved full, sustained recovery from GBS. While she continued to demonstrate moderate anti-dsDNA antibodies and high-level anti-Smith and Sjogren antibodies, C3, C4 and urine readings quickly normalized with no other manifestations of lupus or lupus nephritis 11 months after the initial assessment. At this time she was maintained with hydroxychloroquine, with ongoing depletion of circulating B cells. To our knowledge, this is only the third pediatric patient reported with SLE-GBS.Conclusions: We report severe GBS as the first, dominant manifestation of pediatric SLE. Our case and a review of the literature reveal that cconventional GBS therapy may not be adequate to treat this rare lupus presentation. SLE should be included in the differential diagnosis of GBS. Importantly, treatment experiences and outcomes of such cases need be reported to inform future treatment recommendations.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 539.1-539
Author(s):  
D. Khalifa ◽  
K. Baccouche ◽  
N. El Amri ◽  
H. Zeglaoui ◽  
E. Bouajina

Background:Libman Sacks endocarditis (LS) is an uncommon sterile endocarditis mostly associated with malignancies. It can also complicate the evolution of systemic lupus erythematosus (SLE) in 1 out of 10 patients after many years of evolution of the disease.Objectives:To raise awareness of the possible rare complications of LS in SLEMethods:We report a case of a complicated Libman sacks endocarditis revealing the diagnosis of SLE.Results:A 53 years old female patient with history of hypertension was admitted to the rheumatology department for polyarthritis since 2 months with fatigue and dyspnea. On physical examination, she had no fever nor cutaneous lesions, her pulse rate was accelerated to 112 per minute and there was no heart murmur. Musculoskeletal examination revealed the presence of arthritis of little joints of the hands and bilateral arthritis of the knees. Biological results showed an accelerated sedimentation rate of 49 mm the first hour, elevated c-reactive protein of 79 mg/l, anemia and lymphopenia. Renal and hepatic functions were both normal. Transthoracic cardiac echocardiography showed no valvulopathy but a 5mm sessile vegetation on the mitral valve. To rule out infectious endocarditis, blood cultures came out negative, serological tests for brucella, parvovirus B19, coxiella brunettii, mononucleosis, were also negative. Culture of knee joint fluid for common bacteria and polymerase chain reaction tests for tuberculosis were also negative. The patient presented rapidly with agitation, cerebellar ataxia, and unilateral paresis. CT-scan showed the presence of ischemic lesions in the lenticular nucleus and the semiovale centers. Lumbar punction showed no abnormalities. She was prescribed large spectrum antibiotics and anti coagulants but with persistant worsening of her neurological state. She was then transferred to the intensive care unit. Antinuclear antibodies, came out by then positive 1:3200 with an homogenous fluorescence and with positive anti nucleosome anti bodies. The diagnosis of LS endocarditis associated with SLE complicated with brain embolisms was confirmed with delay.Conclusion:LS endocarditis is not common but when it presents it is often associated with high morbidity and mortality. Health care professionals should consider LS diagnosis in patients with underlying SLE and should be aware of the risk of embolisation. Tests to rule out infectious disease may delay initiation of appropriate treatement leading to severe prognosis. The treatment of LS endocarditis is still not well codified.References:[1]Ibrahim AM, Siddique MS. Libman Sacks Endocarditis. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020 [cité 27 janv 2020]. Disponible sur:http://www.ncbi.nlm.nih.gov/books/NBK532864/[2]Gómez-Larrambe N, González-Tabarés L, Millán B, Cobelo C, Armesto V, Pousa M, et al. Libman-Sacks’ endocarditis: A frequently unnoticed complication. Nefrol Engl Ed. 1 mars 2017;37(2):217‑9.Disclosure of Interests:None declared


2021 ◽  
Vol 23 (1) ◽  
Author(s):  
W. Benjamin Nowell ◽  
Kelly Gavigan ◽  
Carol L. Kannowski ◽  
Zhihong Cai ◽  
Theresa Hunter ◽  
...  

Abstract Background Patient-reported outcomes (PROs) are increasingly used to track symptoms and to assess disease activity, quality of life, and treatment effectiveness. It is therefore important to understand which PROs patients with rheumatic and musculoskeletal disease consider most important to track for disease management. Methods Adult US patients within the ArthritisPower registry with ankylosing spondylitis, fibromyalgia syndrome, osteoarthritis, osteoporosis, psoriatic arthritis, rheumatoid arthritis, and systemic lupus erythematosus were invited to select between 3 and 10 PRO symptom measures they felt were important to digitally track for their condition via the ArthritisPower app. Over the next 3 months, participants (pts) were given the option to continue tracking their previously selected measures or to remove/add measures at 3 subsequent monthly time points (month [m] 1, m2, m3). At m3, pts prioritized up to 5 measures. Measures were rank-ordered, summed, and weighted based on pts rating to produce a summary score for each PRO measure. Results Among pts who completed initial selection of PRO assessments at baseline (N = 253), 140 pts confirmed or changed PRO selections across m1–3 within the specified monthly time window (28 days ± 7). PROs ranked as most important for tracking were PROMIS Fatigue, Physical Function, Pain Intensity, Pain Interference, Duration of Morning Joint Stiffness, and Sleep Disturbance. Patient’s preferences regarding the importance of these PROs were stable over time. Conclusion The symptoms that rheumatology patients prioritized for longitudinal tracking using a smartphone app were fatigue, physical function, pain, and morning joint stiffness.


Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_1) ◽  
Author(s):  
May Nwe Lwin ◽  
Christopher Holroyd ◽  
Dinny Wallis ◽  
Saul Faust ◽  
Hans De Graaf ◽  
...  

Abstract Background/Aims  The coronavirus disease-2019 (COVID-19) caused by the severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) is responsible for over 120,000 UK deaths. Those with chronic inflammatory conditions or receiving immunosuppressive medications are at higher risk of COVID-19 than the general population. As a result, rheumatology patients taking b- or ts-DMARDs were advised to shield. We planned to observe COVID-19 related symptoms and anxiety levels reported by rheumatology patients during the pandemic. Methods  From April 2020, 1,004 rheumatology patients from an advanced therapy database were invited to participate in the adult ImmunoCOVID study to collect daily symptoms (fever, cough, shortness of breath (SOB), sore throat, blocked nose, red-eye, headache, fatigue, joint pain, muscle pain, chills, nausea, diarrhoea and vomiting, loss of senses) and anxiety level using an online portal. Loss of senses were not recorded until week 7 as these were not officially recognized at the pandemic onset. Results  153 patients (rheumatoid arthritis, n = 75, psoriatic arthritis, n = 28, Axial spondyloarthropathy, n = 24, systemic lupus erythematosus, n = 2 and other connective tissue diseases, n = 24) consented and participated. By week 25, 142 patients remained. Among those, 36.57% (±6.09%) (average (±SD)) reported no symptoms over the 25 week period. The main symptoms reported were joint pain (mean=47.94%) followed by fatigue (27.17%). Few patients reported fever (0.94%), cough (8.34%), SOB (4.53%), or loss of senses (1.11%) with more symptoms reported during the first 8 weeks (April/May 2020) and another increase in September/October 2020. The anxiety score (pragmatic 10-point scale) mean (±SD) was 5.60 (±0.34) and remained elevated throughout the study though higher when lockdown began. Conclusion  During the first peak of SARS-CoV-2, the number of patients reporting COVID-19 symptoms appeared high and was associated with high levels of anxiety. As only a small number have been swab-tested, this may suggest that larger numbers of untested individuals have had COVID-19 with mild symptoms. Features of inflammatory rheumatic illnesses may mimic COVID-19 symptoms and create diagnostic difficulty (joint pain and fatigue) whilst anxiety may lead to over-reporting of symptoms in the absence of infection. The key symptoms of fever, cough and SOB were less common and may be most reliable. Disclosure  M. Lwin: None. C. Holroyd: None. D. Wallis: None. S. Faust: None. H. De Graaf: None. C.J. Edwards: Honoraria; Abbvie, Biogen, BMS, Celgene, Fresenius, GSK, Janssen, Lilly, Mundipharma, Pfizer, Roche, Sanofi, UCB. Member of speakers’ bureau; Abbvie, Biogen, BMS, Celgene, Janssen, Lilly, Sanofi, Pfizer, Roche. Grants/research support; Abbvie, Biogen, Pfizer. P051 Table 1:patient reported symptoms and anxiety score from immunoCOVID studyWeek & (number of participants)Fever (%)Cough (%)SOB (%)Joint pain (%)Fatigue (%)Loss of senses (%)No symptoms (%)Tested (n)Test positive (n)Anxiety score1 (26)3.857.6911.5446.1530.77NA30.77006.312 (42)2.3311.639.3052.3834.88NA28.57005.833 (69)1.4514.494.3552.1737.68NA23.19415.884 (92)1.0911.966.5254.3531.52NA27.17206.225 (110)0.0011.716.3145.9533.33NA30.00006.156 (108)0.0010.193.7050.0026.85NA34.26205.747 (119)0.8410.084.2049.5828.57NA34.45205.938 (120)0.007.505.0051.6734.170.8329.17305.629 (124)0.817.263.2352.4229.840.8136.29405.6410 (118)0.008.473.3948.3129.660.8534.75205.2811 (116)0.858.476.7849.1529.661.6933.62305.6512 (131)0.006.114.5856.4926.720.7635.11205.4513 (110)0.916.362.7350.0029.091.8242.73105.4414 (121)0.837.442.4847.1125.620.8339.67805.2815 (100)1.007.003.0046.0023.001.0041.00405.4816 (114)0.887.893.5139.4725.441.7542.98905.2717 (105)0.008.573.8144.7622.860.9543.81425.1018 (107)0.006.543.7443.9319.630.9343.93405.3019 (99)0.005.052.0240.4019.191.0145.45505.0820 (110)0.914.552.7350.9124.550.9139.09NA0Missing data21 (106)0.946.602.8350.0020.750.9439.62405.2822 (104)2.889.626.7349.0430.770.9635.58305.5023 (106)1.897.553.7742.4526.420.9436.79805.8924 (108)0.938.332.7844.4422.220.9341.67605.6125 (94)1.067.454.2641.4915.962.1344.68605.49Average0.948.344.5347.9427.171.1136.575.60SD0.972.312.254.395.350.426.090.34Weekly data are the average of daily reported symptoms and anxiety levels.


2021 ◽  
Vol 8 (1) ◽  
pp. e000474
Author(s):  
Henrik Christian Bidstrup Leffers ◽  
Anne Troldborg ◽  
Anne Voss ◽  
Salome Kristensen ◽  
Jesper Lindhardsen ◽  
...  

ObjectivesSLE displays large clinical heterogeneity that beyond genetic factors may be determined by environmental exposures. In this Danish nationwide study, we aimed to determine if clinical subsets of SLE were associated with smoking history.MethodsAt each of six participating centres, incident or prevalent inpatients and outpatients with SLE were consecutively included. Manifestations forming the basis of SLE classification were registered in an electronic chart system. Patients also provided questionnaire-based data on environmental exposures, including smoking history. Hierarchical cluster analysis was conducted to determine and characterise subsets of patients with similar traits of disease manifestations. Levels of smoking exposure by pack-years were correlated to the identified SLE subsets, as well as discrete SLE manifestations.ResultsThe cohort consisted of 485 patients (88% women and 92% Caucasian) with SLE of which 51% were ever smokers. Common disease manifestations comprised non-erosive arthritis (81%), malar rash (57%), lymphopenia (55%), photosensitivity (50%) and persistent proteinuria (41%). We identified three distinct phenotypic clusters characterised by their preponderance of (A) neurological, serosal and mucosal involvement; (B) renal, haematological and immunological disorders; and (C) acute and chronic skin manifestations. Cluster B was the youngest and had the lowest level of smoking exposure. Age-adjusted regression analyses showed that compared with never smokers a smoking history of >20 pack-years was associated with neurological disorder (OR=3.16), discoid rash (OR=2.22), photosensitivity (OR=2.19) and inversely with haematological disorder (OR=0.40), renal disorder (OR=0.40) and non-erosive arthritis (OR=0.45), p<0.05 for all.ConclusionsOur findings support that SLE presents in varying clinical phenotypes and suggest that they may have differentiated associations with smoking history.


Rheumatology ◽  
2021 ◽  
Author(s):  
Julius Lindblom ◽  
Alvaro Gomez ◽  
Alexander Borg ◽  
Sharzad Emamikia ◽  
Dimitris Ladakis ◽  
...  

Abstract Objectives To investigate the discriminative ability of EQ-5D-3L full health state (FHS) in clinical trials of SLE, and identify factors associated with FHS after treatment. Methods Data from the BLISS-52 (NCT00424476) and BLISS-76 (NCT00410384) trials of belimumab (N = 1684) were utilised. FHS was defined as a response of no problems in all five EQ-5D-3L dimensions, yielding an index score of 1. The Pearson’s chi-square or Fisher’s exact test was employed for comparisons, and logistic regression for adjustments and assessment of independence. Results We demonstrated higher EQ-5D-3L FHS frequencies among patients given standard therapy (ST) plus the licensed belimumab dose versus ST alone (26.1% versus 19.4%; P = 0.001; week 52), and within SRI-4 responders versus non-responders (27.0% versus 19.8%; P &lt; 0.001; week 52) from week 36 to 52. In multivariable regression analysis, SLEDAI-2K (OR: 0.90; 95% CI: 0.87 − 0.94; P &lt; 0.001) and SLICC/ACR Damage Index (OR: 0.79; 95% CI: 0.69 − 0.91; P = 0.001) scores were independently associated with lower FHS frequencies at week 52, while adding monthly infusions of belimumab 10 mg/kg to ST favoured FHS perception (OR: 1.60; 95% CI: 1.15 − 2.24; P = 0.006). Add-on belimumab 10 mg/kg yielded higher FHS frequencies in antimalarial users versus non-users (29.9% versus 20.1%; P = 0.011), and in anti-dsDNA and anti-Sm positive versus negative patients (31.4% versus 13.4%; P &lt; 0.001 and 33.0% versus 22.6%; P = 0.010, respectively), whereas no significant differences were observed in patients given ST alone. Conclusion EQ-5D-3L FHS distinguished belimumab from placebo and responders from non-responders, and exhibited known-group validity in subgroup analysis. FHS may prove a useful patient-reported outcome in SLE studies.


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