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Interleukin 10 (IL-10) plays a role in inflammation and cell-type responses. The anti-SS-A/Ro antibody contributes to leucopenia, and cutaneous and neonatal lupus. Objectives: To evaluate the association between serum IL-10 levels and autoantibodies, disease activity and organ involvement in systemic lupus erythematosus (SLE) patients. Patients and methods: We studied 200 SLE patients and 50 controls. We analyzed organ involvement, disease activity, serum IL-10 and interleukin-6 (IL-6) levels, and antinuclear and antiphospholipid antibody profiles. Results: Serum IL-10 and IL-6 levels were higher in SLE patients than in controls (all p < 0.00001). Serum IL-10 levels were positively correlated with IL-6 (p < 0.00001), CRP (p < 0.00001), fibrinogen (p = 0.003), and ESR (p < 0.00001), and negatively correlated with hemoglobin (p = 0.0004) and lymphocytes (p = 0.01). Serum IL-6 levels were positively correlated with CRP (p < 0.00001), fibrinogen (p = 0.001), and ESR (p < 0.00001); and negatively correlated with hemoglobin (p = 0.008) and lymphocytes (p = 0.03). Elevated serum IL-10 levels were associated with an increased risk of anti-SS-A/Ro antibody positivity (p = 0.03). Elevated serum IL-6 levels were associated with an increased risk of heart (p = 0.007) and lung (p = 0.04) involvement. Conclusions: In SLE patients, increased serum IL-10 levels were associated with increased disease activity and risk of anti–SS-A/Ro antibody positivity.

Hypereosinophilic syndrome (HES) is a rare haematologic disorder characterised by unexplained, persistent eosinophilia with organ involvement. We report the case of a 74-year-old patient who presented with a pemphigoid-like bullous rash as the only manifestation of an HES. This report highlights the importance of the dermatological manifestations of this potentially life-threating condition that may initially present as a skin-limited disease. Bullous pemphigoid (BP) is a very common autoimmune blistering dermatosis that may share common clinical and histological features or associate with an HES. HES should be considered when patients diagnosed with BP demonstrate atypical course under corticosteroids and/or recalcitrant blood eosinophilia.

To identify discrete clusters of systemic lupus erythematosus (SLE) patients based on symptoms and investigate differences across clusters.Data were collected in the US and 5 European countries via the Adelphi Real World Lupus Disease Specific Programme, a cross-sectional survey. Rheumatologists provided data for 5 consecutively consulting adult patients with SLE, who were invited to participate. Identified SLE symptoms were reduced to factors based on commonly concurrent symptoms, using principal-component factor analysis. Factors were used as covariates in a latent-class cluster analysis to identify discrete patient clusters. Patient-reported outcomes and physician-reported data were compared across clusters.Among 1,376 patients, 87% were female and 74% were White. We identified 4 patient clusters (very mild, mild, moderate, and severe) based on 39 signs/symptoms. Physician-reported symptom burden, organ involvement, disease activity, and the number of flares increased with increasing cluster severity (P 0.0001). Patient-reported impact (health status, fatigue, work productivity impairment, anxiety/depression, and emotional impact) increased with increasing cluster severity (P 0.0001). Glucocorticoid and immunosuppressant use increased, and antimalarial use decreased, with increasing cluster severity. In all clusters,20% of patients received biologics;15% of patients not receiving biologics were considered eligible for treatment by their physician. The proportion of physicians and patients satisfied with treatment decreased with increasing cluster severity (P 0.0001).Our large, international, real-world survey of SLE patients and physicians demonstrated strong associations between increased impairment, organ involvement, and humanistic burden in SLE, highlighting an unmet need for effective treatment options in patients with high disease activity.

Hydatid disease or echinococcosis is a tapeworm infection that spreads by contact with animal contaminated faeces. Dogs, sheep, wolves and jackals are definitive hosts passing the eggs of these parasites in their faeces. Humans are accidental, intermediate hosts and become infected after ingesting

IgG4-related sclerosing cholangitis (IgG4-SC) can be classified into four types based on cholangiographic findings and regions of biliary stricture. This cholangiographic classification is useful to differentiate IgG4-SC from mimickers including cholangiocarcinoma, primary sclerosing cholangitis, and pancreatic cancer. Autoimmune pancreatitis (AIP) is a valuable clue for the diagnosis of IgG4-SC because the two are frequently found in association with each other. Two sets of diagnostic criteria for IgG4-SC have been proposed. In Japan, the clinical diagnostic criteria 2020 were recently developed. These clinical diagnostic criteria include narrowing of the intrahepatic and/or extrahepatic bile duct, thickening of the bile duct wall, serological findings, pathological findings, other organ involvement, and effectiveness of steroid therapy. When these criteria are applied, IgG4-SC is initially classified as associated or not associated with AIP, and cholangiographic classification is used for differential diagnosis. In most instances, IgG4-SC can be diagnosed on the basis of clinical diagnostic criteria. However, it is challenging to diagnose isolated IgG4-SC or IgG4-SC not associated with AIP. Here, we review the classification and diagnostic criteria for IgG4-SC, specifically focusing on the clinical diagnostic criteria 2020 and a large IgG4-SC case series from a nationwide survey in Japan.

© International League of Associations for Rheumatology (ILAR) 2021, Evidence for the role of sex in the clinical manifestations of systemic sclerosis (SSc) patients is emerging. Some multicenter cohorts have shown that male SSc patients have more severe disease and worse survival. To assess the differences in clinical manifestations and survival in Portuguese SSc patients according to gender. Data from male and female adult SSc patients included in the Rheumatic Diseases Portuguese Register (Reuma.pt) were analysed and compared. Survival was calculated for patients included in Reuma.pt. within the first two years of diagnosis (inception cohort). In total, 1054 adult patients with SSc were included, 12.5% males. No differences in demographic features and comorbidities were found between the sexes, except for a higher rate of cigarette smokers among men. Diffuse cutaneous SSc and anti-topoisomerase antibodies were more prevalent in males than females. Additionally, male patients presented significantly more myositis, interstitial lung disease and gastric involvement. There were no differences in the patterns of drug use between the sexes. During follow-up, more deaths were reported in men than women (12.1% vs 7.3%, p = 0.04). The overall 1-, 3-, and 5-year survivals from diagnosis of the inception cohort (N = 469) for men vs women were 96.4% vs 98.2%, 93% vs 95.9%, and 75.8% vs 93.2%, respectively, with statistically significant differences (p < 0.01). This study confirms the existence of gender differences in clinical and immunological SSc features. Although SSc is less common in men than women, men have a more severe expression of skin and internal organ involvement and worse survival. Key Points • There are differences in SSc disease manifestations between sexes. • Males more commonly have diffuse cutaneous SSc, anti-topoisomerase antibodies, pulmonary and musculoskeletal involvement. • In the inception cohort, men had worse survival rates than women.

Zusammenfassung Hintergrund Die zystische Fibrose (CF) ist eine multisystemische progrediente Stoffwechselerkrankung mit vorwiegend abdomineller und pulmonaler Beteiligung. Schmerzen sind für Betroffene ein weiteres komplexes und von den Behandlern unterschätztes Problem. Methoden Eine Literaturrecherche deutschsprachiger Leitlinien und englischsprachiger Studien zum Thema CF und Schmerzen wurde durchgeführt, zusätzlich die Beobachtungen zur Diagnostik und Therapie eines CF-Patienten mit progredienten thorakalen Schmerzen ausgewertet. Ergebnisse Die Recherche ergab, dass zur Diagnostik und Therapie thorakaler Schmerzen bei CF keine deutschsprachigen Leitlinien oder Konsenspapiere existieren. Die europäischen und amerikanischen Erhebungen zeigen aber die große Relevanz des Themas und postulieren einen Zusammenhang von Schmerzintensität mit einer erhöhten Mortalität. Sie enthalten jedoch keine Daten zur Effektivität der Schmerztherapie. Anhand dieser Daten und des Fallberichts eines jungen CF-Patienten mit stärksten Thoraxschmerzen bei pulmonalen Exazerbationen lassen sich die CF-spezifischen Herausforderungen der Schmerztherapie illustrieren. Neben den Schmerzen an sich sind auch Analgetika angesichts der multiplen Organdysfunktionen mit besonderen Risiken wie gastrointestinalen Blutungen, opioidinduzierter Atemdepression oder opioidinduzierter Obstipation verbunden. Diskussion Schmerztherapie bei Patienten mit zystischer Fibrose und Multiorganbeteiligung erfordert ein sorgfältiges Monitoring und interdisziplinäres Handeln. Empfehlungen zum Schmerzmanagement sollten in die deutschsprachigen CF-Leitlinien aufgenommen werden.

Objective: Decreased maximal mouth opening is a common and disabling manifestation in systemic sclerosis patients. We aimed to study the course of maximal mouth opening, determinants of smaller maximal mouth opening over time and the burden of smaller maximal mouth opening on mouth handicap. Methods: Consecutive systemic sclerosis patients participating in the prospective Leiden Combined Care in systemic sclerosis cohort were included. Annual clinical assessment included maximal mouth opening measurement and mouth handicap evaluation (Mouth Handicap in Systemic Sclerosis scale). Presence of microstomia (maximal mouth opening Results: A total of 382 systemic sclerosis patients were studied with median follow-up time of 2.0 years (interquartile range = 0.0–3.0). At baseline, mean maximal mouth opening was 42.2 ± 8.0 mm and 7% suffered from microstomia. Annual decrease of > 5.0 mm in maximal mouth opening during follow-up occurred in 63 patients and was accompanied by increase in disease severity. Disease characteristics at baseline independently predictive for smaller maximal mouth opening over time were: more extended skin subtype; peripheral vasculopathy; pulmonary, renal and gastrointestinal involvement. Smaller maximal mouth opening was significantly associated with more reported mouth handicap. Conclusion: The course of maximal mouth opening is stable in a majority of systemic sclerosis patients. Still, maximal mouth opening over time was smaller in patients with more severe organ involvement. Although microstomia was infrequent, a smaller maximal mouth opening was significantly associated with more mouth handicap, indicating the importance to address maximal mouth opening in routine care of systemic sclerosis patients.

Background: Inflammatory rheumatic diseases (IRD) are often associated with the involvement of various organs. However, data regarding organ manifestation and organ spread are rare. To close this knowledge gap, this cross-sectional study was initiated to evaluate the extent of solid organ manifestations in newly diagnosed IRD patients, and to present a structured systematic organ screening algorithm. Materials and Methods: The study included 84 patients (63 women, 21 men) with newly diagnosed IRD. None of the patients received any rheumatic therapy. All patients underwent a standardised organ screening programme encompassing a basic screening (including lungs, heart, kidneys, and gastrointestinal tract) and an additional systematic screening (nose and throat, central and peripheral nervous system) on the basis of clinical, laboratory, and immunological findings. Results: Represented were patients with connective tissue diseases (CTD) (72.6%), small-vessel vasculitis (16.7%), and myositis (10.7%). In total, 39 participants (46.5%) had one or more organ manifestation(s) (one organ, 29.7%; two organs, 10.7%; ≥three organs, 6.0%). The most frequently involved organs were the lungs (34.5%), heart (11.9%), and kidneys (8.3%). Lastly, a diagnostic algorithm for organ manifestation was applied. Conclusion: One-half of the patients presented with a solid organ involvement at initial diagnosis of IRD. Thus, in contrast to what has been described in the literature, organ manifestations were already present in a high proportion of patients at the time of diagnosis of IRD rather than after several years of disease. Therefore, in IRD patients, systematic organ screening is essential for treatment decisions.

Recurrence of DRESS syndrome is poorly characterized, and dermatologists must be prepared to predict, identify, and manage patients after treatment of the initial presentation. In this study, a primary literature search was conducted using PubMed, capturing all articles recording cases of DRESS syndrome recurrence. Forty-two articles were included for review comprising a total of 60 patients. The average age of patients was 46.3 years and time to recurrence was 123 days. Recurrent episodes presented more frequently with a higher fever and eosinophil absolute peak. Relapse was most often attributed to the introduction of a new medication (n = 18). Of the 17 cases in which outcome data were available, the survival rate of those experiencing recurrence was 71%. Viral reactivation with HHV-6 and organ involvement of the liver were frequently recorded complications. In essence, viral reactivation, severe internal organ involvement, and hematological abnormalities all portended a poorer prognosis in those experiencing DRESS syndrome recurrence. An adequate course of treatment should be maintained until clinical and laboratory parameters normalize, with a slow taper to minimize the likelihood of relapse in those most at risk.

Henoch-Schonlein purpura (HSP) is the most common childhood systemic vasculitis. The present study aims to investigate the effectiveness of the immature granulocyte (IG) percentage as a new marker for predicting internal organ involvement in HSP. This study included 75 patients aged below 18 years who were diagnosed with HSP. The mean age was 7.48±2.77 years. The male/female ratio was 1.14. The findings showed that 35 (46.7%) of the patients had an internal organ involvement. The mean IG percentage was 0.88±0.68 among the patient group with HSP internal organ involvement, while it was 0.31±0.15 in the group without internal organ involvement, and a significant difference was determined between the 2 groups (P=0.000). The findings showed that the patients with renal involvement had the highest mean IG percentage (IG; 1.00±0.21). When the cutoff value for the IG percentage was specified as 0.45 to predict internal organ involvement, the sensitivity was 77.1%, and the specificity was 85%. In this study, the findings showed that IG percentage increased among patients with internal organ involvement in HSP and that its sensitivity, specificity, and predictive values were higher in predicting internal organ involvement compared with other markers.

According to the American Urological Association/American Society of Clinical Oncology/American Society for Radiation Oncology/Society of Urologic Oncology Guideline on treatment of nonmetastatic muscle invasive bladder cancer (MIBC), females requiring radical cystectomy (RC) should undergo concomitant anterior pelvic exenteration despite low rates of malignant involvement of gynecologic organs. We present the clinicopathological characteristics of patients with MIBC treated with neoadjuvant chemotherapy (NAC) and evaluate the impact of NAC on gynecologic organ involvement.An institutional review board approved review of patients with cT2-T3 MIBC treated with RC at our institution between 2005 and 2018 was performed. Patients were stratified by receipt of NAC.A total of 186 females with cT2-T3 MIBC underwent RC during the study period, of whom 67.7% received NAC prior to RC. Patients who received NAC were more likely to have cT3 disease, preoperative hydronephrosis, and variant histology on transurethral resection (p0.001, p=0.004, p=0.029, respectively). Rates of recurrence or metastasis were similar between groups (27.0% vs 26.7%, p=0.964). No patients had isolated genitourinary organ recurrence (median followup 32.1 months). Nine patients (5.7%) had gynecologic organ involvement (6 NAC vs 3 no NAC, p=0.978). Among those who underwent hysterectomy, 2 patients (3.1%) who received NAC had uterine involvement compared to none in the no NAC cohort (p=0.551). Rates of vaginal involvement were similar between the groups (4 NAC vs 3 no NAC, p=0.402). Additionally, 1 patient who received NAC had incidentally diagnosed localized endometrial cancer. No women had fallopian tube or ovarian involvement.Even among high risk patients with MIBC, gynecologic organ involvement of MIBC is rare, and organ preservation, especially of the ovaries, is likely safe.

We assessed the role of [18F]FDG-PET/CT in identifying and managing cancer of unknown primary site (CUP syndrome). We reviewed [18F]FDG-PET/CT scans of individuals with CUP syndrome recorded in clinical referral letters from 2012 to 2019. We evaluated the identification of primary tumor (PT) by [18F]FDG-PET/CT, according to histological subtype, and the impact on clinical management. The median age was 65 years, 36/64 males (56%). PTs were detected in 28/64 (44%) patients. Detection was significantly lower in patients with squamous cell carcinoma (SCC) than with other histologies combined, p = 0.034. Mean age, mean SUVmax (10.6 ± 6.0) and organ involvement were similar between patients with and without discovered PTs; and between patients with SCC and with other histologies combined. However, those with SCC were less likely than the others to present with multi-lesion involvement, p

Background: The first case of COVID 19 illness was detected on 31st December 2019 and the disease has progressed globally causing significant morbidity and mortality. The disease initially thought to be a respiratory virus soon showed manifestations involving other systems and diagnosis and treatment of the disease became more complicated. This study aims to derive a scoring system based on health records of patients suffering from COVID 19, to help in early triaging of the illness and therefore allowing for early institution of treatment. Aims and Objective: To establish a scoring system inclusive of clinical, laboratory and radiological parameters to assist in the prognosis of patients afflicted with COVID 19 illness. Materials and Methods: Health records of 138 COVID patients has been included in the study. The scoring system comprises of parameters including Age, Co-morbidities, Shortness of breath, Saturation, Pulse Rate, Respiratory rate, temperature, D dimer, Neutrophil Lymphocyte ratio, Troponin I, Organ involvement, Radiology. The cumulative scoring ranges from 0-16. The mortality rate among the subjects included was 25.4%. Results: All parameters involved were found to be independent risk factors for mortality. Patients were effectively categorized based on the scoring system and mortality found to be associated with increasing scores. This model displayed good discrimination (AUC =0.875) and the sensitivity and specificity of the model was found to be 0.857 and 0.767 respectively. Conclusion: This scoring system has been designed to categorize based on the systemic involvement of the disease and thus would serve as a reliable indicator for prognostic assessment in patients. [ABSTRACT FROM AUTHOR] Copyright of Asian Journal of Medical Sciences is the property of Manipal Colleges of Medical Sciences and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a severe drug-induced hypersensitivity reaction characterized by skin rash, fever, blood abnormalities, and multiple organ involvement. The diagnosis of DRESS syndrome is often delayed because of its variable presentation. DRESS syndrome induced by antituberculosis drugs has rarely been reported. A 45-year-old female patient taking anti-tubercular drugs since 2 weeks presented to our hospital with extensive skin lesion with mucosal involvement. The case responded well to treatment with systemic corticosteroids and withdrawal of isoniazid with no recurrence of lesions and further follow-up.

Sarcoidosis may present with many rheumatological symptoms as well as mimic and/or may occur concomitantly with many other rheumatic diseases. We examined the demographic, clinical and laboratory characteristics of patients diagnosed with sarcoidosis in the rheumatology department. This study planned as retrospective cross-sectional study. Medical records of patients who applied to our rheumatology outpatient clinic due to complain of musculoskeletal problems and then diagnosed sarcoidosis were retrospectively investigated. Joint findings, extrapulmonary involvements, and coexisting rheumatic disease were evaluated. Fifty-six patients (41.21 +/- 7.83 years, 75% female) were included. The duration of the disease was 49.61 +/- 29.11 months, and the follow-up period was 26.66 +/- 13.26 months. All patients had pulmonary system involvement. Arthralgia was present in 91.10% of 56 patients and arthritis in 89.29% of patients. Examining the subtypes of the arthritis findings, mono-arthritis was found in 31/50 (62%) patients, oligo-arthritis in 15/50 (30%) patients, and polyarthritis in 4/50 (8%) patients. A total of 11 (19.60%) patients were diagnosed with uveitis. Excision of the mediastinal LAP was performed in a total of 37 patients (66.1%) and became the most commonly employed method. Considering the treatment distribution of the patients under followed-up, it is seen that non-steroidal anti-inflammatory treatments were used in 15 (26.8%) patients, corticosteroids in a total of 40 (71.4%) patients, methotrexate in a total of 15 patients (26.8%), azathioprine in six (10.7%) patients, hydroxychloroquine in 14 (25%) patients, and infliximab in one (1.8%) patient. As sarcoidosis is a mimicking disease, a good differential diagnosis should be made to avoid misdiagnosis and in order not to be late in diagnosis and treatment. Physicians, especially rheumatologists, should remember sarcoidosis more frequently as the disease may overlap with other rheumatological diseases and may occur with many rheumatological manifestations.

Objectives: To evaluate the demographic and clinical characteristics of patients with Behcet's syndrome (BS), and to define their associations with the presence of major organ involvement (MOI).Method: Medical records of 2118 patients (964 males, 1154 females) were analysed retrospectively. MOI was defined as the presence of at least one of vascular, eye, nervous, or gastrointestinal system involvement. Univariable and multivariable binary and ordinal logistic regression analyses were applied to assess the factors that were potentially associated with MOI.Results: The mean ± sd age at diagnosis was 30.5 ± 9.4 years. Genital ulcer and joint involvement were more common in females (both p < 0.001), while MOI was more frequent in males (p < 0.001). Genital ulcer (p < 0.001) and vascular involvement (p = 0.006) were more common in patients with a younger age at diagnosis, while joint involvement was more common in older patients. A total of 1097 patients (51.8%) had at least one MOI, 322 (15.2%) at least two MOIs, and 48 (2.3%) at least three MOIs. Male gender, smoking history, and absence of genital ulcer were significantly associated with MOI in multivariable binary logistic regression. Multivariable ordinal regression analyses confirmed the association between MOI and male gender and smoking, but not the protective effect of genital ulcers. In both regression analyses, we found no significant effects of age, human leucocyte antigen-B51, skin involvement, or joint involvement on MOI.Conclusion: Male gender and positive smoking history have a significant influence on the presence of MOI in patients with BS.

Sarcoidosis is a systemic granulomatous disease with heterogenous clinical manifestations. Here we review the diagnosis of sarcoidosis and propose a clinically feasible diagnostic work-up and monitoring protocol. As sarcoidosis is a systemic disease, a multidisciplinary approach is recommended for best outcomes. However, since the lungs are frequently involved, the pulmonologist is often the referral physician for diagnosis and management. When sarcoidosis is suspected, diagnosis needs to be confirmed and organ involvement/impairment assessed. This process is also required to establish whether the patient is likely to benefit from treatment, as many cases of sarcoidosis are self-limited and remit spontaneously. Whether or not treatment is started, effective regular follow-up is necessary to monitor changes in the disease, including extension, progression, remissions, flare-ups, and complications.

Sarcoidosis is a disease that can affect a multitude of organs and manifest as cutaneous disease. Cutaneous manifestations of sarcoidosis vary widely in morphology, earning the nickname of "great imitator," and the diagnosis often requires clinicopathologic correlation and additional laboratory and radiographic workup. We present the case of a 42-year-old African American woman with ichthyosiform sarcoidosis on the bilateral lower extremities. As one of the rarest specific variants of cutaneous sarcoidosis, ichthyosiform is understudied and has been primarily documented in case reports and series. We undertake a comprehensive review of the literature to identify key clinicopathologic features including the characterization of sites of cutaneous and systemic involvement, as well as typical histopathological findings. Lower extremities were the most involved body site (85.7%). Extracutaneous organ involvement centered around pulmonary (65.7%), lymph node (57.1%), and ocular (31.4%) involvement. Of the histopathological features reported, hyperkeratosis (51.4%) and diminished stratum granulosum (62.9%) were most frequently reported in conjunction with dermal granulomas (100%). We hope that these findings will serve to aid clinicopathological correlation and accurate diagnosis of ichthyosiform sarcoidosis.

Introduction:Cardiac involvement is rare in hydatid cyst disease, which accounted for 0.5%-2% of all hydatidosis cases. Cardiac cysts usually occur as part of a wider infestation with extracardiac involvement. This study aimed to describe the general characteristics, imaging findings, and range of organ involvement in cases of cardiac hydatid disease.Methods:Retrospective assessment of cardiac hydatid disease records, between 2007 and 2019, was undertaken to identify patients with cardiac hydatidosis. Hydatid cysts were assessed by transthoracic echocardiography, magnetic resonance imaging, and computed tomography. Clinical symptoms, treatment modalities, and follow-up were also evaluated.Results:Over the study period, 22 (13 males: 59.1%; mean age: 43.1 years; range: 12-63 years) patients with 24 cardiac hydatid cysts were identified. The most common symptom was chest pain, which occurred in 13 (59.1%) patients. Cardiac cysts were located in the left ventricle (n=10, 41.7%), right ventricle (n=5, 20.8%), interventricular septum (n=5, 20.8%), and pericardium (n=4, 16.7%). Extracardiac involvement was present in 14 (63.6%) patients, most commonly affecting the liver (n=10, 45.4%), but in 8 (36.3%) patients, there was no extracardiac organ involvement. Cardiac hydatid cysts were surgically removed in 20 (90.9%) patients.Conclusion:Cardiac hydatid disease is very uncommon, with the left ventricle as the most commonly affected structure in this series. Chest pain was the most common presenting symptom. Extracardiac involvement is common, so patients with cardiac hydatid cysts should be investigated for involvement of other organs.

A 40-year-old Japanese woman developed malignant-phase hypertension complicated by thrombotic microangiopathy, progressing to end-stage renal disease. Five years later, she was diagnosed with pulmonary arterial hypertension and interstitial pneumonia. Despite a lack of overt skin sclerosis, nucleolar staining in our indirect immunofluorescence analysis and nailfold capillaroscopy facilitated the diagnosis of anti-PM/Scl antibody-positive systemic sclerosis. We observed the persistent presence of anti-PM/Scl antibodies throughout the clinical course, suggesting that her kidney disease was scleroderma renal crisis. Anti-PM/Scl antibodies can be associated with multiple organ diseases. Careful attention to a patient's antinuclear antibody pattern and dermatological findings may help clarify the etiology of undiagnosed diseases.

If blistering occurs in childhood, the possibility of hereditary epidermolysis bullosa should be considered even if the symptoms are mild. Besides clinical and histological examination, molecular genetic screening is diagnostically relevant. For localized forms, symptomatic, topical therapy options are currently still the primary choice. Of particular interest is the new option of topical therapy with diacerein 1 % cream. In the case of a pronounced clinical picture with extracutaneous organ involvement, multidisciplinary management is required. In the future, new forms of therapy such as autologous epidermal stem cell transplantation and gene therapeutic procedures may be applied. Human genetic counselling is indispensable.

Amaç: Geniş bir klinik spektruma neden olan Brusellozda ağrı kaçınılmaz bir semptomdur. Bu çalışmada Bruselloz tanılı hastalarda ağrının varlığı, şiddeti, lokalizasyonu, ağrı kesici kullanımı ve yaşam kalitesi üzerine etkisini inceledik.Gereç ve Yöntemler: Klinik ve laboratuvar bulgularına göre Bruselloz tanısı alan hastalar çalışmaya dahil edildi. Hastaların yaş, cinsiyet, Brusellozun klinik evresi, organ tutulumu, serum C-reaktif protein (CRP) düzeyleri ve standart tüp aglütinasyon testi (STA) titresi kaydedildi. Skorlar, Kısa Ağrı Envanteri (BPI) ve Dünya Sağlık Örgütü Yaşam Kalitesi Ölçeği Kısa Form Türkçe Versiyonu (WHOQOL-BREF-TR) ile hastalarla yüz yüze seanslarda elde edildi.Bulgular: Çalışmaya dahil edilen 92 Brusella hastasının medyan yaşı 40.5 idi. Hastaların %54.3'ü erkek, %45.7'si kadındı. Başvuru sırasında %57,6'sı akut, %31.5'i subakut ve %10,9'u kronik Bruselloz idi. Hastaların %31.5'inde organ tutulumu mevcuttu. Tanı anında hastaların %89,1'inde ağrı şikayeti vardı. Ağrı lokalizasyonu en sık sırasıyla diz, sırt, kalça ve omuzdaydı. Hastaların %51.1'i günde en az bir kez nonsteroid antiinflamatuar ilaç kullanıyordu. Yaş, hastalık evresi, organ tutulumu, serum CRP düzeyi ve STA titresi ile WHOQOL-BREF ve VAS ölçümü değerleri arasında istatistiksel olarak anlamlı ilişki bulunmadı. WHOQOL-BREF Psikolojik (p=0.003) ve WHOQOL-BREF Sosyal (p=0.008) ölçümü değerleri kadınlarda erkeklere göre daha yüksek bulunduSonuç: Ağrı Bruselloz hastalarında sık görülen bir semptomdur. Laboratuvar parametreleri ve organ tutulumu ne olursa olsun Bruselloz hastalarının ağrıları hastalığın tüm evrelerinde değerlendirilmelidir. Ağrı semptomları bu hasta grubunda yaşam kalitesini etkiler ve sık ağrı kesici kullanımına neden olarak istenmeyen yan etkilere neden olabilir., Objective: Pain is an inevitable symptom in Brucellosis, which causes a wide clinical spectrum. In this study, we aimed to examine the effect of pain on presence, severity, localization, analgesic intake and quality of life in patients diagnosed with Brucella.Materail and Methods: Patients diagnosed with Brucella based on clinical and laboratory findings were included in the study. Age, gender, clinical stage of Brucella, organ involvement, serum C-reactive protein (CRP) level and standard tube agglutination test (STA) titer of the patients were recorded. The Brief Pain Inventory (BPI) and the Turkish Version of the World Health Organization Quality of Life Scale Short Form (WHOQOL-BREF-TR) were administered face-to-face to the patients.Results: The median age of 92 Brucella patients included in the study was 40.5 years. 54.3% of the patients were male and 45.7% were female. At admission, 57.6% were acute, 31.5% subacute, and 10.9% chronic. Organ involvement was present in 31.5% of the patients. At the time of diagnosis, 89.1% of the patients had pain complaints. Pain localization was most frequently in the knee, back, hip and shoulder, respectively. 51.1% of the patients were using nonsteroidal anti-inflammatory drugs (NSAIDs) at least once a day. There was no statistically significant correlation between age, disease stage, organ involvement, serum CRP level and STA titer and WHOQOL-BREF and VAS measurement values. WHOQOL-BREF Psychological (p=0.003) and WHOQOL-BREF Social (p=0.008) measurement values were found to be higher in women than men.Discussion and Conclusion : Pain is a common symptom in Brucella patients. Regardless of age, laboratory parameters and organ involvement, pain in Brucella patients should be questioned at all clinical stages. It affects the quality of life and may cause undesirable side effects by causing frequent painkiller intake.

Hypocomplementemic urticarial vasculitis syndrome (HVUS) belongs to the group of systemic small-vessel vasculitis and is characterized by persistent urticarial lesions lasting more than 24 hours, hypocomplementemia and frequent coexistence of organ involvement. Lungs, kidneys, joints, gastrointestinal tract, eyes and central nervous system may be involved in the course of the condition. The diagnosis of HUVS is based on the diagnostic criteria – fulfilment of both major criteria (the presence of chronic urticarial lesions with hypocomplementemia) and at least two minor criteria. HUVS may be associated with other systemic conditions. The diagnosis of the syndrome may also precede the development of systemic lupus erythematosus (SLE) in both the paediatric and adult populations. Due to the rarity of the disease, no randomized clinical trials are assessing the effectiveness of individual therapeutic options, and recommendations for the management of HUVS are based on case reports. Glucocorticosteroids (GCSs) are most commonly used as monotherapy or in combination with cyclosporine A, methotrexate, azathioprine, cyclophosphamide or mycophenolate mofetil. In the paper, the clinical picture, recommended diagnostics and therapeutic options for HUVS are discussed.

Systemic scleroderma has inflammation and fibrosis playing a crucial role and eventually leading to severe functional failure and damage of multiple organs. Three primary mechanisms are mainly involved in the pathogenesis of scleroderma: vascular anomalies, excess fibrosis, and autoimmune phenomenon. The present case study shows a severe cardiac dysfunction with low cardiac output syndrome (LCOS) with cardiac, hepatic and renal impairment, peripheral ischemia, neurologic symptoms associated or even aggravated by a probable severe toxicity to azathioprine therapy. Currently there is no cure for systemic sclerosis, the aims of treatment are- as recommended also by EULAR: to relieve symptoms, to prevent the condition from progressing, as much as possible, to detect and treat complications early and to minimize any disability.

Incidence of ANCA antibodies in patients with systemic lupus erythematosus (SLE) is described in 24-31 %, but they are not related to the distribution and severity of organ involvement in SLE; the routine monitoring is not recommended. Overlap syndrome of systemic lupus erythematosus and ANCA associated vasculitis (AAV) is rare. The difficult diagnosis and treatment of this syndrome is described in this case report of the patient with SLE and severe kidney involvement resulting from AAV.

Background Interstitial lung disease (ILD) comprises a heterogeneous group of disorders characterized by multifocal diffuse lung involvement. Similarly, COVID-19 has varied multispectral organ involvement. Patients with underlying ILD and coexistent COVID-19 infection may lead to an acute blow to the already deceased lung. Case presentation A 58-year-old man presented with fever and cough with expectoration for the past 4 days associated with breathlessness. He was a smoker and alcoholic for the past 20 years. His saturation was low in room air around 84% and improved to 98% with 10 l/min of nasal oxygen. Further investigation shows acute deterioration of ILD. Conclusion COVID-19 infection has a spectrum of manifestations. Acute deterioration of ILD is rarely reported in the literature. Etiology should be investigated further.

Graphical abstract, Highlights • Cardiac echinococcosis is a rare but potentially fatal manifestation of hydatid disease. • Antiparasitic agents with surgical cyst excision is the treatment of choice. • Heart team decision-based management is warranted in cases of other organ involvement.

Klotho long recognized for its role in anti-aging, is potentially implicated in the pathogenesis of rheumatoid arthritis, systemic lupus erythematosus, and systemic sclerosis. Aging of the immune system coincides with the inability of the body to recognize self-antigens, which often leads to autoimmune responses. The role of Klotho in these autoimmune diseases should be of high interest; however, few articles have been published exploring the role of Klotho in the pathogenesis, organ involvement, or clinical manifestation of rheumatoid arthritis, systemic lupus erythematosus, and systemic sclerosis. Herein, we discuss information gathered from peer-reviewed publications to describe the emerging role of Kl in these select rheumatologic autoimmune diseases.