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The American Journal of Case Reports May 2021BACKGROUND Systemic lupus erythematosus (SLE) is a systemic autoimmune disease resulting from dysregulation of the immune response. In genetically predisposed subjects,...
BACKGROUND Systemic lupus erythematosus (SLE) is a systemic autoimmune disease resulting from dysregulation of the immune response. In genetically predisposed subjects, infections reputedly trigger an immune activation leading to autoimmunity and overt autoimmune diseases such as SLE. CASE REPORT We report the case of a 19-year-old woman who presented to our hospital reporting high-grade fever, dry cough, and polyarthralgia despite a course of empiric antibiotic and steroid therapy administered by her general practitioner (GP). On physical examination, she had a malar rash, a palpable erythematous maculopapular non-itchy rash over the limbs and trunk, and mild polyarthritis. A contrast computed tomography (CT) scan of the chest showed a pulmonary right upper-lobe consolidation with air bronchogram and multiple necrotizing conglomerate mediastinal lymph nodes. Culturing of collected samples from endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) of the mediastinal lymph node revealed growth of Mycobacterium kansasii. Antinuclear antibodies (ANA) and lupus anticoagulant (LAC) were positive. A diagnosis of M. kansasii infection associated with SLE was made. She was started on anti-mycobacterial and hydroxychloroquine therapy and entered into a joint rheumatological and infectious disease follow-up. Six months later, a CT scan with positron emission tomography (PET) showed a significant reduction in size of the basal right upper-lobe consolidation and hypermetabolic activity in multiple pulmonary areas and mediastinal lymph nodes. ANA and LAC tests were repeated and remained positive. The decision was made to continue the ongoing therapy course for 1 year in total. CONCLUSIONS Clinical and experimental studies have suggested the association of mycobacterial infections with SLE and as a possible infectious trigger of autoimmunity. We describe a unique case of M. kansasii infection associated with the onset of SLE in a young woman.
Topics: Adult; Endoscopic Ultrasound-Guided Fine Needle Aspiration; Female; Humans; Lung Neoplasms; Lupus Erythematosus, Systemic; Lymph Nodes; Mediastinum; Mycobacterium kansasii; Young Adult
PubMed: 34006819
DOI: 10.12659/AJCR.929866 -
Medicine May 2015The authors aimed to explore whether distinct clinical, serological, and urinalysis findings are associated with specific histological classes of lupus nephritis....
The authors aimed to explore whether distinct clinical, serological, and urinalysis findings are associated with specific histological classes of lupus nephritis. Clinical and laboratory features were recorded at the time of clinical diagnosis from 297 consecutive patients with biopsy-confirmed lupus nephritis. Univariate and logistic regression analyses were performed and a risk score was developed to estimate the risk for developing different classes of lupus nephritis. Variables independently associated with class II included absence of malar rash, negative anti-dsDNA, and ≤5 urine leucocytes/high power field (hpf); with III/IV: age at nephritis diagnosis ≤32 years old, presence of musculoskeletal features, new-onset hypertension, positive anti-dsDNA, >5 urine leucocytes/hpf, creatinine >1.2 mg/dL, cellular casts >1/hpf, and absence of nephrotic range proteinuria; with V: age at nephritis diagnosis >32 years, malar rash, absence of musculoskeletal complaints or serum C3 hypocomplementemia, nephrotic range proteinuria, and ≤9 urine erythrocytes/hpf. A risk predictive score of specific histological classes was calculated for each patient. Associations between 2, 3 or more risk factors with specific histological classes were also revealed [Odds ratios (95% confidence interval) (≥2 risk factors) was 6.7 (2.8-17.4) for class II nephritis, 15.6 (5.1-47.8), and 8.2 (3.6-19.0) for classes III/IV and for class V, respectively (≥3 risk factors)]. The identification of independent factors associated with specific classes of lupus nephritis can provide guidance in selecting specific therapeutic modalities, particularly in cases in which renal biopsy is contraindicated.
Topics: Adult; Age Factors; Biopsy; Creatinine; Exanthema; Female; Hematuria; Humans; Lupus Nephritis; Male; Middle Aged; Musculoskeletal Diseases; Odds Ratio; Photosensitivity Disorders; Proteinuria; Risk Factors; Sex Factors
PubMed: 26020385
DOI: 10.1097/MD.0000000000000829 -
Medicine Jan 2010Ethnicity and environmental factors could be involved in the heterogeneity of systemic lupus erythematosus (SLE). We conducted this study to define clinical and...
Ethnicity and environmental factors could be involved in the heterogeneity of systemic lupus erythematosus (SLE). We conducted this study to define clinical and serologic correlations and autoantibody clusters in SLE patients in South China. We retrospectively reviewed the records of 917 patients with SLE admitted to our hospital between January 2005 and June 2008. We found the following associations between autoantibodies and clinical manifestations to be statistically significant: anti-double-stranded DNA (anti-dsDNA) with higher prevalence of renal disorder, leukopenia, and anemia; anti-Sm with higher prevalence of malar rash/discoid rash, pericarditis, and leukopenia; anti-ribonucleoprotein (anti-RNP) with higher prevalence of Raynaud phenomenon and photosensitivity; anti-deoxyribonucleoprotein (anti-DNP) with higher prevalence of arthritis and lower prevalence of renal disorder; anti-Scl-70 with higher prevalence of anemia and Raynaud phenomenon; anti-Jo-1 with higher prevalence of pericarditis; and anti-centromere with higher prevalence of Raynaud phenomenon. Three autoantibody clusters were identified: Cluster 1 (anti-Ro, anti-Sm, and anti-RNP [Ro/Sm/RNP], with a significantly lower percentage of elderly SLE and higher prevalence of photosensitivity, malar rash/discoid rash, Raynaud phenomenon, and leukopenia); Cluster 2 (anti-Ro [Ro], with a lower percentage of pediatric SLE); and Cluster 3 (the absence of anti-extractable nuclear antigen antibodies [ENA ve], with a lower percentage of adult SLE and lower prevalence of alopecia). In summary, this study not only confirms both anti-dsDNA and anti-Sm as specific markers for classifying SLE, but also demonstrates that photosensitivity is not associated with anti-Ro but with anti-RNP, and a negative association is found between renal disorder and anti-DNP in patients in South China. These results are different from results found in other populations. The higher prevalence of anti-dsDNA and renal disorder results in less difference in the prevalence of anti-dsDNA and renal disorder among the 3 autoantibody clusters in SLE patients in South China, which could be related to ethnicity and widespread industrial pollution in South China.
Topics: Adolescent; Adult; Autoantibodies; China; Female; Humans; Lupus Erythematosus, Systemic; Male; Middle Aged; Retrospective Studies; Risk Factors
PubMed: 20075706
DOI: 10.1097/MD.0b013e3181cb449c -
Indian Dermatology Online Journal Jan 2011Treatment of collagen vascular diseases like systemic sclerosis, dermatomyositis, systemic lupus erythematosus (SLE) and even overlap syndromes has been difficult since...
BACKGROUND
Treatment of collagen vascular diseases like systemic sclerosis, dermatomyositis, systemic lupus erythematosus (SLE) and even overlap syndromes has been difficult since long. Monumental success of dexamethasone-cyclophosphamide pulse (DCP) in pemphigus has prompted many a dermatologist to try it in other autoimmune diseases.
MATERIALS AND METHODS
DCP was given as per standard regimen for six to nine pulses. Immunosuppressives were given for 12-18 months in dermatomyositis, SLE, and overlap syndrome, and for 12 months in systemic sclerosis. Daily dose of steroid was tapered off gradually.
RESULTS
The treatment resulted in 90% improvement in skin binding in systemic sclerosis, 80% in exertional dyspnea, 40% in dysphagia, but minimum improvement was seen in Raynauds and digital tip ulcerations. No improvement in pigmentation was noted. In SLE, malar rash cleared in 70%, joint pain in 80%, oral ulcerations reduced in 80%, fever in 98%, and photosensitivity improved in one-third of patients. In dermatomyositis, improvement in muscle tenderness was seen in 100%, improvement in proximal myopathy and heliotrope rash in 80%, and improvement of shawl sign was observed in 80% of the patients. Some flattening of Gottron papules and plaques was noted in some patients. Both overlap patients improved significantly. Out of 24 patients, three were lost to follow-up, one resorted to homeopathic medicine and two expired (one dermatomyositis, one SLE). Side effects like hypertension, hyperglycemia, pyoderma, fungal infections, obesity, psychosis, etc. were seen in 25-30% of patients.
CONCLUSIONS
We conclude that DCP is relatively safe, effective as well as cheap compared to methylprednisolone pulse. Side effects are also less compared to daily regimen of steroids. We also observed that patients who reported early and put on pulse early responded better.
PubMed: 23130206
DOI: 10.4103/2229-5178.79858 -
Clinical Case Reports Apr 2023Considering the clinical polymorphism of the disease, longstanding skin lesions located on the face, resembling erysipelas in an endemic area should always be...
Considering the clinical polymorphism of the disease, longstanding skin lesions located on the face, resembling erysipelas in an endemic area should always be investigated for CL and thus, atypical presentations should be kept in mind.
PubMed: 37020670
DOI: 10.1002/ccr3.7163 -
Pediatric Rheumatology Online Journal Jun 2021Dermatomyositis with positive anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody has a distinct phenotype associated with small hand joint arthritis,... (Review)
Review
Adolescent-onset anti-MDA5 antibody-positive juvenile dermatomyositis with rapidly progressive interstitial lung disease and spontaneous pneumomediastinum: a case report and literature review.
BACKGROUND
Dermatomyositis with positive anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody has a distinct phenotype associated with small hand joint arthritis, mucocutaneous ulceration, palmar papules and less muscle involvement. It is also associated with increased risk of rapidly progressive interstitial lung disease (RP-ILD) and has a high mortality rate in adults. There is evidence that cases complicated with spontaneous pneumomediastinum (PNM) have an increase in mortality. While most of the evidence for this rare disease is derived from the adult literature, we report a case diagnosed in an adolescent complicated with both RP-ILD and PNM with a good outcome after aggressive immunosuppressive therapy. Our case also illustrates the potential challenges in diagnosis of this condition in the setting of non-specific clinical manifestations, the need for a high index of suspicion, and the importance of testing for myositis-specific antibodies (MSA) early to aid in diagnosis given the risk of rapid progression in these patients.
CASE PRESENTATION
A 16-year-old Chinese female presented with fever and cough for 1 day, and finger swelling for 3 weeks. Physical examination revealed arthritis of fingers and wrists, ulcers and palmar papules over fingers, hyperpigmentation of interphalangeal joints, and rash over the neck. The diagnosis of dermatomyositis was made 1 month later with the onset of malar rash, Gottron's papules, calcinosis and myalgia. The diagnosis was supported by the presence of anti-MDA5 antibody and evidence of inflammatory myopathy on magnetic resonance imaging. In retrospect, she already had interstitial lung disease at first presentation manifested as cough and opacity on chest radiograph, which was later confirmed with chest computed tomography. She was treated according to adult guidelines with steroid and calcineurin inhibitor. Her disease was resistant to initial therapy and was complicated by RP-ILD and spontaneous PNM. Intensive immunosuppressive therapy including cyclophosphamide and rituximab were required to induce remission.
CONCLUSIONS
Recognition of distinct clinical features of anti-MDA5 antibody-positive dermatomyositis and testing for MSA is crucial in patients with skin ulceration and abnormal pulmonary findings of unknown etiology, as prompt diagnosis with early aggressive treatment and anticipation of complications could make a difference in the outcome of this disease with high mortality.
Topics: Adolescent; Age of Onset; Autoantibodies; Dermatomyositis; Disease Progression; Female; Humans; Interferon-Induced Helicase, IFIH1; Lung Diseases, Interstitial; Mediastinal Emphysema; Time Factors
PubMed: 34193210
DOI: 10.1186/s12969-021-00595-1 -
Archives of Rheumatology Jun 2019This study aims to investigate the positive detection rate and predictive value of autoantibodies, including anti-double stranded deoxyribonucleic acid (anti-dsDNA)...
OBJECTIVES
This study aims to investigate the positive detection rate and predictive value of autoantibodies, including anti-double stranded deoxyribonucleic acid (anti-dsDNA) antibodies, anti-histone antibodies (AHAs), anti-ribosomal (anti-Rib) P antibodies, anti-Smith (anti-Sm) antibodies, anti-U1 ribonucleoprotein (anti-U1RNP) antibodies, anti-Sjögren's syndrome type A antibodies and anti-Sjögren's syndrome type B antibodies, on organ damage in patients with systemic lupus erythematous (SLE).
PATIENTS AND METHODS
A total of 225 patients with SLE (37 males, 188 females; mean age 37.4±15.9 years; range, 7 to 80 years) were evaluated retrospectively. Statistical analysis was performed to obtain the positive detection rate of autoantibodies and to investigate the predictive value.
RESULTS
There were statistically significant differences of positive anti-dsDNA antibodies in renal damage, photosensitization, hematological abnormalities and serositis (p<0.05) and a statistically significant difference of positive AHAs in photosensitization (p<0.05). There was statistically significant difference of positive anti-U1RNP antibodies in renal damage (p<0.05). There were also statistically significant differences of positive anti-Smith antibodies in renal damage, arthritis, photosensitization, oral ulcers, hematological abnormalities and serositis (p<0.05) and of positive anti-Rib antibodies in renal damage, arthritis, photosensitization, malar rash, hematological abnormalities and serositis (p<0.05). However, there were no statistically significant differences of positive anti-Sjögren's syndrome type B antibodies and anti-Sjögren's syndrome type A antibodies in renal damage, arthritis, malar rash, neuropsychiatric disorders, hematological abnormalities and serositis (p>0.05).
CONCLUSION
Autoantibody spectrum is an important serological basis for SLE diagnosis. There are differences in the autoantibodies distribution of SLE patients with different organ damage, suggesting a certain clinical value for prediction of organ damage in SLE.
PubMed: 31497762
DOI: 10.5606/ArchRheumatol.2019.6981 -
The Journal of Rheumatology Apr 2012Male patients with systemic lupus erythematosus (SLE) are thought to be similar to female patients with SLE, but key clinical characteristics may differ. Comparisons... (Comparative Study)
Comparative Study
OBJECTIVE
Male patients with systemic lupus erythematosus (SLE) are thought to be similar to female patients with SLE, but key clinical characteristics may differ. Comparisons were made between male and female patients with SLE in the Hopkins Lupus Cohort.
METHODS
A total of 1979 patients in the Hopkins Lupus Cohort were included in the analysis.
RESULTS
The cohort consisted of 157 men (66.2% white, 33.8% African American) and 1822 women (59.8% white, 40.2% African American). The mean followup was 6.02 years (range 0-23.73). Men were more likely than women to have disability, hypertension, thrombosis, and renal, hematological, and serological manifestations. Men were more likely to be diagnosed at an older age and to have a lower education level. Women were more likely to have malar rash, photosensitivity, oral ulcers, alopecia, Raynaud's phenomenon, or arthralgia. Men were more likely than women to have experienced end organ damage including neuropsychiatric, renal, cardiovascular, peripheral vascular disease, and myocardial infarction, and to have died. In general, differences between males and females were more numerous and striking in whites, especially with respect to lupus nephritis, abnormal serologies, and thrombosis.
CONCLUSION
Our study suggests that there are major clinical differences between male and female patients with SLE. Differences between male and female patients also depend on ethnicity. Future SLE studies will need to consider both ethnicity and gender to understand these differences.
Topics: Adult; Aged; Cohort Studies; Comorbidity; Female; Follow-Up Studies; Humans; Lupus Erythematosus, Systemic; Male; Middle Aged; Racial Groups; Sex Characteristics; Young Adult
PubMed: 22382348
DOI: 10.3899/jrheum.111061 -
Contemporary Clinical Dentistry Jul 2012Rashes can occur in any part of the body. But rash which appears on face has got both psychological and cosmetic effect on the patient. Rashes on face can sometimes be...
Rashes can occur in any part of the body. But rash which appears on face has got both psychological and cosmetic effect on the patient. Rashes on face can sometimes be very challenging to physicians and dermatologists and those associated with oral manifestations pose a challenge to dentists. Butterfly rash is a red flat facial rash involving the malar region bilaterally and the bridge of the nose. The presence of a butterfly rash is generally a sign of lupus erythematosus (LE), but it can also include a plethora of conditions. The case presented here is of a female with butterfly rash along with typical bright red discoloration of gingiva. The clinical, histopathological and biochemical investigations suggested the presence of rosacea.
PubMed: 23293500
DOI: 10.4103/0976-237X.103637 -
Journal of Family Medicine and Primary... Dec 2022Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune disease of unknown etiology, mainly affecting female of child-bearing age group. Clinical...
OBJECTIVES
Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune disease of unknown etiology, mainly affecting female of child-bearing age group. Clinical scenario of SLE is not well defined in east region of India, especially in tribal region of Jharkhand. This article is mainly focused on clinical and laboratory characteristics of SLE in tribal region of Jharkhand.
MATERIALS AND METHODS
This was an analytical cross-sectional single-centered study conducted at RIMS, Ranchi, a tertiary care center of Jharkhand, between November 2020 and October 2021. A total 50 patients were diagnosed as SLE based on Systemic Lupus International Collaborating Clinics criteria.
RESULTS
Forty-five (90%) of patients in our study were female, with female to male ratio of 9:1. The mean age of presentation was 26.78 ± 8.12. Constitutional symptoms were found in 96% of patients, followed by anemia in 90% of patients. Renal involvement was found in 74% of patients, followed by polyarthritis (72%), malar rash (60%), and neurological manifestations (40%). Anti-nuclear antibody, anti-dsDNA, and anti-Smith antibodies were found positive in 100%, 84%, and 80% of patients, respectively.
CONCLUSIONS
Clinical characteristics of SLE as per our study would help the health care professionals in this region to identify the disease at early stage and initiate appropriate treatment.
PubMed: 36994038
DOI: 10.4103/jfmpc.jfmpc_848_22