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1.
Case Rep Rheumatol ; 2024: 3123953, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38774817

RESUMO

Background: Generalized morphea is a rare fibrosing skin illness that progresses from erythematous, violet-colored skin patches to sclerotic plaques. Another uncommon immune-mediated connective tissue disease called eosinophilic fasciitis (EF) evolves to cause sclerosis and woody skin induration. The coexistence of the two is extremely rare and has a poorer prognosis. Our case report is one of the first to report burn injuries as a trigger factor for EF and generalized morphea overlap. Case Presentation. A 36-year-old man presented with acute onset of rapidly progressing skin thickening, tender edema, and skin contractures involving all extremities, shortly after enduring burn injuries from a gasoline explosion. Workup was remarkable for peripheral eosinophilia, hypergammaglobulinemia, and elevated C-reactive protein. Skin biopsy demonstrated sclerodermoid changes and sclerotic thickening of subcutaneous fibrous septa associated with stromal mucin, dermal perivascular, diffuse lymphoplasmacytic infiltrate with eosinophils, decreased CD34 expression, and increased factor XIIIa. He was subsequently diagnosed with an overlap of generalized morphea and eosinophilic fasciitis. The patient had only limited improvement with steroids, methotrexate, mycophenolate mofetil, and intralesional triamcinolone acetonide injections. Conclusion: Generalized morphea with concomitant EF indicates some degree of therapeutic resistance and poor prognosis with a low quality of life. Burn injuries can be a trigger factor for this overlap syndrome. Prompt identification of at-risk individuals and initiating aggressive management are necessary.

2.
Clin Rheumatol ; 43(1): 59-65, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37980305

RESUMO

OBJECTIVE: Neuromyelitis optica (NMO), also known as Devic's disease, is a rare inflammatory demyelinating disorder causing myelitis and optic neuritis. While there have been reports of systemic lupus erythematosus (SLE) and primary Sjogren's syndrome (SS) occurring with NMO, a formal association is not established. We aimed to investigate the occurrence of NMO in SLE and SS patients and study the clinical characteristics and outcomes of NMO and SLE/SS hospitalizations utilizing the national inpatient sample (NIS) database. METHODS: The NIS database from 2016 to 2019 was used to extract data. Adult hospitalizations with the principal or secondary diagnosis of NMO were included. We classified NMO patients with and without concomitant diagnosis of SLE or Sjogren's syndrome. We evaluated and compared the clinical characteristics and outcomes of NMO hospitalizations with and without SLE or Sjogren's syndrome. STATA17 was used for data analysis. We also calculated the odds ratio of NMO in SLE and Sjogren's syndrome. RESULTS: There were a total of 16,360 adult hospitalizations with the principal or secondary discharge diagnosis of NMO. Among all NMO hospitalizations, 1425 (8.71%) had the primary or secondary diagnosis of SLE or SS. The odds of NMO in SLE and Sjogren's syndrome were noted to be 12.29 and 5.56, respectively. NMO with SLE/SS group had higher proportion of females (89.82% vs 79%, P value < 0.001), African Americans (56.63% vs 38.28, P value < 0.001), and Asians (5.73% vs 3.25, P value 0.04). The Charlson comorbidity index was higher for NMO-SLE/SS overlap (2.44 vs 1.28, P value < 0.001). There was no significant difference in overall mortality rates of both groups (2.11% vs 1.2%, P value 0.197). There were significantly higher reported seizures (14.73% vs 6.05, P value < 0.001) and paraplegia (21.75% vs 13.93%, P value < 0.001) in NMO-SLE/SS patients. These patients also had a longer length of stay in comparison to the reference group (7 vs 5 days, P value < 0.001) as well as higher total charges. CONCLUSIONS: NMO patients had a 12-fold higher risk of SLE and 5-fold higher risk of Sjogren's disease when compared to general population. Patients with overlap of NMO and SLE or Sjogren's were predominantly women and were more likely to be African-American. Co-existence of these autoimmune disorders was associated with poor prognosis in terms of higher morbidity for patients and increased health care burden. Key Points • NMO is a rare autoimmune disease seen predominantly in women in the middle age group with low overall mortality. • SLE and Sjogren's have increased odds of NMO in comparison to general population. • NMO patients have high rates of several complications such as paraplegia, quadriplegia, seizures, blindness, sepsis, and respiratory failure with even higher rates of seizures and paraplegia in those with concomitant SLE or Sjogren's.


Assuntos
Doenças Autoimunes , Lúpus Eritematoso Sistêmico , Neuromielite Óptica , Síndrome de Sjogren , Adulto , Pessoa de Meia-Idade , Humanos , Feminino , Masculino , Síndrome de Sjogren/complicações , Síndrome de Sjogren/epidemiologia , Neuromielite Óptica/complicações , Neuromielite Óptica/epidemiologia , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/epidemiologia , Doenças Autoimunes/complicações , Convulsões/complicações , Paraplegia/complicações
3.
Cureus ; 15(6): e39947, 2023 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-37283596

RESUMO

Cardiac tamponade is an uncommon complication of systemic sclerosis (SSc) with a high mortality rate. Here, we report a case of a 58-year-old patient with limited cutaneous systemic sclerosis (lcSSc), gastroesophageal reflux disease (GERD), diabetes mellitus, pulmonary hypertension (PHTN), and COVID-19 infection, which occurred one month ago, presenting with a large hemorrhagic pericardial effusion and early cardiac tamponade. The patient had an acute onset of progressive dyspnea and anasarca. On examination, she was tachypneic, tachycardic, desaturating on room air, and hypotensive. Pitting edema up to thighs and bilateral basilar crackles were also appreciated. Labs were remarkable for negative troponin, chest X-ray with pulmonary congestion, D-dimer at 6.01, CT angiogram negative, brain natriuretic peptide level at 73 pg/mL, C-reactive protein level at 7.64 mg/dL, normal complement levels, and negative COVID-19 test results. Echocardiography showed early tamponade and a large circumferential effusion with chamber collapse. Right heart catheterization was performed finding PHTN at 54 mmHg. Pericardiocentesis drained 500 mL of the hemorrhagic effusion. Fluid analysis showed RBC at 220,000/uL, WBC at 5000/uL, protein 4.8 g/dL, lactate dehydrogenase level of 1275 U/L, and negative cytology. The patient was treated for serositis from lcSSc flare with mycophenolate mofetil and steroids, and responded very well. Hemorrhagic cardiac tamponade is a very rare phenomenon in limited scleroderma. A recent COVID-19 infection could have served as a trigger factor for our patient's lcSSc in long remission to flare up. Clinicians should maintain a high index of suspicion and a low threshold for intervention when lcSSc patients have an acute onset of cardiac compromise, especially with a history of a recent COVID-19 infection.

4.
Case Rep Rheumatol ; 2023: 8083212, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37350776

RESUMO

Background: Gout is an inflammatory arthritis caused by monosodium urate (MSU) deposition. Acute gout is a dramatic painful swelling of the joint; however, MSU can deposit in other tissues as well, including skin, gastrointestinal tract, and bones over time. Disseminated tophi in the skin are a rare presentation of gout known as gout nodulosis. We present a case of gout nodulosis with subcutaneous diffuse miliary nodules in nonarticular areas with concurrent findings suggestive of chronic inflammatory arthritis. Case Presentation. A 39-year-old patient presented with intermittent painful swelling in multiple joints with prolonged morning stiffness. On exam, synovitis was present in multiple proximal interphalangeal joints, wrists, elbows, and knees. Chronic raised pearly nodular rash and swellings on extensor aspects of arms, legs, and anterior abdomen were noticeable. He had negative rheumatoid factor and anti-CCP antibody, C-reactive protein of 0.23 mg/dL, erythrocyte sedimentation rate of 37 mm/hr, and uric acid of 10.6 mg/dL. Hand X-rays revealed severe periarticular osteopenia and joint space narrowing in several joints. Musculoskeletal ultrasound showed a double contour sign at multiple joints and a tophaceous deposit over the olecranon fossa. The biopsy of the nodular rash was consistent with tophi. He was diagnosed with chronic tophaceous gout with skin nodulosis and possible overlap of seronegative rheumatoid arthritis given his X-ray findings. Conclusion: This case discusses one of the rare presentations of gout with disseminated gouty tophi in the skin to raise clinical awareness. The clinical dilemma of the overlap of gout and rheumatoid arthritis posing a diagnostic challenge for clinicians is also highlighted.

7.
Cureus ; 10(9): e3284, 2018 Sep 11.
Artigo em Inglês | MEDLINE | ID: mdl-30443454

RESUMO

Contrast-induced acute kidney injury, also called contrast-induced nephropathy, is one of the main causes of acute renal failure/acute kidney injury (AKI) in hospitalized patients within 48 to 72 hours of contrast media administration during various radiologic procedures. Several factors can be responsible for contrast-induced acute tubular necrosis (ATN); however, patient and procedure-related factors play the lead role in determining the development of contrast-induced nephropathy. There is no definitive treatment and hydration remains the mainstay preventive strategy. This article will review the incidence, criteria for definitive diagnosis, and an effective approach on how to prevent contrast-induced nephropathy in a clinical setup.

8.
Cureus ; 10(9): e3288, 2018 Sep 11.
Artigo em Inglês | MEDLINE | ID: mdl-30443458

RESUMO

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with varied natural history and multisystemic involvement. The pathogenesis is multifactorial and complex precipitating the formation of autoantibodies. One of the main factors in SLE is the interaction between environmental triggers and genetic factors. Genome-wide association study technology has led to the identification of more than 80 loci which produce key proteins that lead to small pathophysiological changes and are associated with SLE. There has been an improvement in the management of the disease with newly standardized scores that have been validated in assessing disease activity and quality of life, and have helped in clinical care as well as research. The last five decades have seen a marked improvement in the prognosis of SLE, thanks to better general care and the development of newer immunosuppressive drugs, more specifically biological agents.

9.
Cureus ; 10(8): e3230, 2018 Aug 29.
Artigo em Inglês | MEDLINE | ID: mdl-30410836

RESUMO

Hypertension is the most prevalent clinical symptom arising from various cardiovascular disorders. Likewise, it is considered a precursor or sequelae to the development of acute coronary artery disease and congestive heath failure (CHF). Hypertension has been considered a cardinal criterion to determine cardiovascular function. According to the World Health Organization (WHO) global observatory data, hypertension causes more than 7.5 million deaths a year, about 12.8% of the total human mortality. Similarly, the Center for Disease Control (CDC) states that 35% of the American adults have been estimated to have a persistently high blood pressure, which makes it about one in every three adults. Hypertension is a modifiable symptom that can be managed through pharmacological and non-pharmacological methods and standard protocols set forth by the American Heart Association (AHA). With new findings from various clinical trials related to the management of hypertension, new developments and recommendations have been made to update the previously established protocols for hypertension. This article aims to discuss and dissect the modern updates of hypertension management as comprehensively elaborated in the 2017 Hypertension Clinical Practice Guidelines.

10.
Cureus ; 10(8): e3129, 2018 Aug 10.
Artigo em Inglês | MEDLINE | ID: mdl-30345188

RESUMO

Neurogenic stunned myocardium (NSM) is defined as the occurrence of cardiac abnormalities due to neurological events such as stroke, seizures, etc. These events lead to dysfunction of the autonomic nervous system (ANS) and ultimately cause injury to the myocardium. The clinical features seen in NSM include elevated troponin level, left ventricular dysfunction, and changes on the electrocardiogram (ECG). However, these features are also seen in Takotsubo cardiomyopathy as well as in an acute coronary syndrome (ACS). Hence, diagnosing the condition by clinical presentation alone is difficult. Thus, a patient of NSM who is at increased risk of developing coronary heart disease may require invasive procedures such as cardiac catheterization to rule out ACS. This review aims at raising awareness about NSM among physicians so that management of patients can be individualized.

11.
Cureus ; 10(8): e3166, 2018 Aug 20.
Artigo em Inglês | MEDLINE | ID: mdl-30357001

RESUMO

In the older population, especially the hospitalized patients who are prone to dehydration and hypovolemia, orthostatic hypotension (OH) presents as a debilitating disease. How different pharmacological and non-pharmacological interventions affect the incapacitating symptoms (falls and episodes of syncope), morbidity, and mortality related to OH has become a topic of debate. OH can predispose to ischemic heart disease (IHD). A non-pharmacological approach consisting of mobilization, early lifestyle changes, and therapeutic maneuvers is the first choice in the management of these patients. Individuals with persistent symptoms require pharmaceutical therapy to increase blood volume and peripheral vascular resistance. This article summarizes the management of OH that is vital to cope with the needs of the growing geriatric populations.

12.
PeerJ ; 3: e1264, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-26557420

RESUMO

Background. There is growing awareness of the public health importance of mental well-being both in the general population and in specific groups. The well-being of health professionals is likely to influence the quality of the care they deliver. This study was carried out to examine the well-being of Pakistani healthcare professionals, and to evaluate the psychometric performance of the Warwick-Edinburgh Mental Well-being Scale (WEMWBS) in this population. Methods. A cross-sectional survey was carried out from June, 2013 to December, 2014 among 1,271 Pakistani health care providers (HCPs) working in seven different cities in Punjab province, Pakistan, to examine the acceptability, internal consistency, test-retest reliability and content and construct validity of the English version of the WEMWBS in a Pakistani population sample. All data were analyzed in SPSS v. 21. Results. Our analysis demonstrated unidimensional construct validity, high internal consistency (0.89) and test-retest reliability, good validity and easy readability of WEMWBS in our sample of Pakistani HCPs. The mean WEMWBS score was 48.1 (SD 9.4), which is lower than in the general population in other countries. Male HCPs scored significantly higher on the WEMWBS than their female counterparts (P < 0.05), and older respondents had higher scores. Conclusion. The WEMWBS appears acceptable for use in Pakistani HCPs, and findings from this study verify its validity and internal consistency for this population sample. Our respondents had lower well-being scores than those reported in general population surveys in the UK.

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