Objective: Pulmonary arterial hypertension (PAH) affects 2.6% of adults and 36% of people with chronic obstructive pulmonary disease (COPD). Several case reports and small case series suggested a hyperthyroidism-PAH association. Design: Retrospective chart review. Methods: We undertook a retrospective chart review (1982–2018) to assess PAH prevalence in a multi-ethnic convenience sample of hyperthyroid adults with multiple etiologies. We calculated associations of pulmonary artery maximum systolic pressure (PSAPmax) with subject age, and maximum serum triiodothyronine (T3) and thyroxine (T4), free T3, and T4, minimum serum thyroid-stimulating hormone (TSH), and thyroid antibody titers, comparing PAH prevalence and the odds of being undiagnosed as to hyperthyroidism etiology by gender and ethnicity/race. Results: We found a high prevalence of PAH in hyperthyroid people, like that reported for people with COPD. We found no significant association between PSAPmax and any thyroid function test or thyroid antibody titer. As reported more recently in the general population, PSAPmax significantly correlated with age in hyperthyroid people. There was no significant disparity in the prevalence of PAH among White, non-Hispanic Black, and Latinx hyperthyroid people or between genders. The percentage of patients whose hyperthyroidism etiology was undiagnosed was high with significant disparity only between non-Hispanic Black and White people and between men and women. PAH was common in hyperthyroid subjects with any hyperthyroidism etiology. Conclusions: 2D-echocardiography should be performed in all hyperthyroid people because PAH is common, especially in older people because of their co-morbidities and poorer prognoses. Further research is needed regarding demographic disparities in being undiagnosed as to hyperthyroidism etiology. Principal Verdicts/Significance Statement: We reconfirmed the high PAH incidence in hyperthyroidism, previously reported, but profoundly under-recognized by physicians, to patients’ detriment. Further, we found that the shift in the general PAH population from younger to older individuals is mirrored in hyperthyroid people with PAH. This is concerning because older people have more co-morbidities and worse prognoses, necessitating early, effective intervention. PAH was present with diverse hyperthyroidism etiologies, suggesting that it is multicausal, resulting from autoimmunity, thyroid hormone excess, and goitrous upper airway obstruction and should be considered, regardless of etiology. Our observations that many subjects had no established hyperthyroidism etiology and that males and Blacks were likelier to be undiagnosed are concerning, warranting further study.
Background: In recent studies, a strong association between rheumatoid arthritis (RA) and chronic periodontal disease (CPD) has been identified, indicating common disease pathogenesis and risk factors. One of them is the presence of the pathogen Porphyromonas gingivalis (PG), which can initiate the process of citrullination, by secreting the enzyme Porphyromonas Peptydil Arginine Deminase (PPAD). The aim of the study is to show the presence of PG in RA patients, and to evaluate the association of the PG presence with anti-citrullinated proteins/peptides (ACPA) positivity i.e. anti CCP and anti MCV positivity. Methods: The study included 80 participants - 30 patients which fulfilled 2010 ACR/EULAR RA classification criteria and 50 controls, which were genetically analyzed for the presence of PG by Chelex®100 method and polymerase chain reaction (PCR) and for the presence of anti CCP and anti MCV autoantibodies with the ELISA method. Results: Twenty out of thirty RA patients (80 %) and 16 out of 50 controls (32%) were positive for PG. (χ 2 = 11.461, p <0.001 for OA and χ2 = 13.91, p <0.001 for HC). Of the PG-positive RA patients, 83% had positive anti-CCP and 79% had positive anti-MCV test. The odds ratios OR of 25 and 19 were statistically significant (p = 0.008 and p =0,014 respectfully). Conclusions: PG was present more frequently in RA patients and there was a statistically significant association with anti CCP and anti MCV antibodies.
Objective: The main objective of this study was to evaluate the association of IL6-174 G/C gene polymorphisms and the response to tocilizumab (TCZ) in patients with systemic juvenile idiopathic arthritis (s-JIA). Methods: Sixty patients with s-JIA (37 males and 23 females with median age at onset of 5.2 years) who received TCZ were recruited. Basic demographic, laboratory and clinical data were collected alongside the IL-6 haplotype status. The overall response to treatment with TCZ was assessed according to a number of variables including the extent of disease activity reduction, the achievement of clinically inactive disease, the necessity to switch to another biologic disease modifying anti-rheumatic drug (bDMARD) and the achievement of a glucocorticoid-free state. Results: Three IL6 -174 genotypes, including, GG, GC, and CC were found with higher frequencies of GC genotype. These genotypes had non-significant association with the response of s-JIA patients to IL-6 blockade in this cohort study. However, a longer time frame from disease onset to diagnosis was associated with poorer long-term treatment response. Conclusion: We observed no significant impact of IL6 -174 G/C gene polymorphisms on treatment response to TCZ in s-JIA Egyptian patients. The observation that a shorted timeframe between symptom onset and diagnosis is associated with better long-term response to TCZ provides evidence for a therapeutic “window of opportunity” in patients with s-JIA.
Nailfold capillaroscopy is currently the best method to investigate microvascular abnormalities in systemic sclerosis and related conditions, and in other rheumatic conditions in which there is a clinical suspicion of microangiopathy. Although easy to perform, it is essential that the operators have been properly trained about correct method of images acquisition and interpretation. There are some parameters to indicate a normal/healthy capillaroscopic picture, but it is important to consider that there is a great variability in the capillary structure both interindividual and intraindividual. The early differential diagnosis between primary and secondary RP is the best advantage that the technique may offer. Remarkable capillaroscopic alterations are found in the majority of cases of systemic sclerosis and the so-called “scleroderma spectrum disorders†(dermatomyositis, mixed connective tissue disease, undifferentiated connective tissue disease). Nevertheless, some capillaroscopic changes have been observed in systemic lupus erythematosus, Sjogren’s syndrome, psoriatic and rheumatoid arthritis. Discussion about controversies on this topic should be encouraged, leading to a progressive development of capillaroscopy as a routine investigation in rheumatology.
Primary biliary cirrhosis (PBC) is an autoimmune cholestatic disease of the liver which affects mainly middle-aged women characterized by progressive destruction and loss of the small intrahepatic bile ducts which in turn, may lead to end-stage liver disease. The typical clinical phenotype is characterized by a middle-aged female with elevated cholestatic enzymes and positive antimitochondrial antibodies (AMA). However, apart from this typical presentation, there are important variants in everyday clinical practice. These variants include the AMA-negative PBC, the isolated AMA positivity, the AMA-positivity in patients with well-established autoimmune hepatitis (AIH), the premature ductopenic PBC variant and the PBC variant with characteristics of AIH (PBC-AIH variant). In this mini-review, we summarize and discuss the literature data and our own experience on the PBC variants highlighting also the uncertainties and a potential new era of the research agenda.
Beginning with our observations in the 1990s that many patients with Type 2 diabetes (T2DM) have clinical signs and symptoms of hyperandrogenism such as hirsutism, male pattern alopecia, acne, menstrual irregularity, and infertility we went on to systematically evaluate these patients for hyperandrogenism and found that, with the notable exception of patients whose Type 2 diabetes developed in the setting of chronic Hepatitis C and no known family history of Type 2 diabetes, all had evidence of non-classic adrenal hyperplasia (NCAH) [1-4]. Subsequently, we found this relationship to be true even in those T2DM patients without clinical features of hyperandrogenism.
We report a 58 yr old lady, who had right temple swelling for 2 years, which on excision biopsy showed features of vasculitis. During the Rheumatology review, Clinical evaluation didn’t reveal any features suggestive of Giant cell arteritis and inflammatory markers were normal. She had localised granulomatous changes without giant cells and pathologist gave the opinion as Kimura’s disease. It has been described in East Asia especially in Japan as Juvenile Giant cell vasculitis (also known as Kimura’s disease (KD). It was reported by Kimura et al . as chronic inflammatory disorder of unknown aetiology with granulomatous changes mimicking vasculitis but no giant cells. It is very rare in Caucasians and early diagnosis will result in appropriate treatment and sparing the immunomodulatory treatment. This case is presented to increase awareness of KD and to highlight the features which may aid the diagnosis.
Background and Aim of the Work : JIA is the commonest rheumatic disease in childhood characterized by inflammatory arthritis lasting more than 6 weeks before the 16 th birthday. In addition to routine ESR and CRP, there are other inflammatory biomarkers as SAA. It is one of the major acute phase reactants which was found to be elevated in inflammatory arthritis and a good indicator of disease activity. This study assessed the value of SAA level in a cohort of patients with JIA. Subjects and Methods : 45 JIA patients and 40 healthy controls were recruited from the outpatient clinic of Rheumatology and Rehabilitation Department at MUCH. All patients underwent a thorough clinical evaluation. Assessment of JIA patients involved assessment of disease activity, tenderness and functional status. Laboratory tests were done including: CBC, ESR, CRP and SAA. Results : A significant rise in SAA levels was found in JIA patients compared to control group and it was significantly higher in SJIA subtype. SAA level was positively correlated with JADAS-27, VAS, physician global assessment, C-HAQ, Ritchie articular index score, platelet count and ESR in 1 st hour and 2 nd hour. The levels of SAA were significantly lower in JIA patients taking methotrexate while it was significantly higher in cyclosporine treated patients. Conclusion : SAA can be used as additional indicator of JIA disease activity. Moreover, it can help in differentiation between subtypes when combined with clinical features of the disease and it may be considered in assessment of patient, s response to therapy.
This is an effort to present to the medical profession, a new concept for evaluating the level of health of an individual, through the theory of Professor George Vithoulkas. The theory of Levels of Health has proven to be a valuable aid to clinicians as it enables them not only to evaluate the patient’s health status, but also to adapt the course of individual treatment. This is achieved by assessing the body's response, to therapy of any kind. When coupled with the ‘Continuum of unified theory of diseases’, disease and treatment are better understood and provide a reference standard for clinicians. The application of the concepts of Psychoneuroimmunology (PNI) and those correlating the suppression of acute diseases with simultaneous emergence of chronic conditions opens up new horizons in understanding the human body's nature in this respect. The treatment of acute diseases can lead in two opposite directions: it can either bring about a cure or, on the contrary, cause a gradual degeneration of the body's PNI defense. With regard to ‘symptoms,’ their reduction or the disappearance following a treatment, is either because the body does not need them anymore, having reached a higher level of health or, that it cannot maintain them anymore, as its health has been degraded due to the treatment. The ideal treatment should not simply eliminate the symptoms while the overall health deteriorates. Instead, it must aim at enhancing the action of the immune system in its own direction by strengthening the symptoms generated by it. This way the immune system becomes stronger after getting rid of the disease and the overall health becomes better.
The aim of this study is to determine the frequencies of abnormal pregnancy outcomes in a cohort of patients with systemic lupus erythematosus (SLE). Data of 69 pregnancies of 37 SLE patients were analyzed retrospectively. Lupus activity was assessed based on SLE Disease Activity Index (SLEDAI) criteria. Compared with pregnancies without Lupus nephritis (LN), pregnancies with LN were associated with a higher risk of still birth (p=0.092), higher rate of eclampsia (p=0.103), intrauterine growth restriction (IUGR) (p=0.556), and pregnancy induced hypertension (PIH) (p=0.412). PIH (17.4% vs 11.1%), IUGR (34.7% vs 11.8%), preterm delivery (26.1 % vs 11.8%), still birth (13% Vs 5.6%) and eclampsia (13% Vs 0%), all were observed to be higher in active lupus patients compared to those in remission. However, these differences were not statistically significant (p>0.05). Absence of LN, proteinuria and low complement component 3 (C3) were potential (p<0.15) predictors for live births. Anti-Ro antibodies, high anti-double stranded DNA antibody (anti-dsDNA), and low C3 were strongly associated with pre-term live births and Anti-Ro antibodies was significantly associated with IUGR. In conclusion SLE in pregnancies in a multinational population in Qatar was associated with higher adverse pregnancy outcomes. Disease activity during pregnancy, proteinuria, LN and eclampsia/preeclampsia were all negatively associated with pregnancy outcome such as IUGR, still births and preterm delivery. Laboratory parameters such as presence of Anti Ro/La antibody and low level of C3 were also associated with adverse pregnancy outcome.
