Capillaroscopic Insights: Exploring the Connection Between Microvascular Changes and Pulmonary Manifestations in Systemic Sclerosis.

BACKGROUND: Systemic sclerosis (SSc) is a complex connective tissue disease characterized by microangiopathy, immune dysregulation, and fibrosis. Early detection of microvascular abnormalities using nailfold videocapillaroscopy (NVC) is crucial in assessing disease progression and associated disease's involvement such as interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH). OBJECTIVE: This study aims to explore the relationships correlation between NVC patterns, clinical manifestations, and systemic complications in SSc. METHODS: We analyzed the data of 63 patients, predominantly female (95%), with a mean age of 49 years and an average disease duration of 42 months. Patients were categorized into early, active, and late patterns based on NVC findings. Clinical features, including digital ulcers (DU), ILD, and PAH, were assessed. Pearson correlation analyses were performed to evaluate the relationships between capillary loss, neoangiogenesis, ILD, and PAH. RESULTS: The early pattern group (mean mRSS 2.36) exhibited minimal microvascular damage and systemic involvement, with no DUs. In the active pattern group (mean mRSS 10.40), 34.38% had diffuse cutaneous SSc (dcSSc), with 15.63% presenting DUs, 65.63% ILD, and 37.5% PAH. The late pattern group (mean mRSS 18.00) showed the most severe disease, with 80% having DUs, 70% dcSSc, 90% ILD, and 70% PAH. Pearson correlation analyses revealed strong correlations between capillary loss and ILD (r = 0.7255) and PAH (r = 0.6369). A moderate correlation was found between neoangiogenesis and PAH (r = 0.5592). CONCLUSION: The study demonstrates that progressive microvascular damage in SSc, as visualized by NVC, correlates strongly with the severity of systemic complications. Early detection of capillary loss and neoangiogenesis using NVC is critical for timely interventions, which could improve patient outcomes by mitigating the progression of ILD and PAH.

Eosinophilic fasciitis: unraveling the clinical tapestry of a rare case and review of literature.

Eosinophilic fasciitis (EF) remains a diagnostic challenge due to its rarity and resemblance to scleroderma. This case report aims to provide a cohesive exploration of EF's clinical nuances, emphasizing the importance of accurate diagnosis and effective management. A 52-year-old male developed bilateral forearm and calf hardening, along with erythema, pruritus, and pain four months prior to the presentation in our Clinic. The symptoms initially debuted bilaterally in the forearms and progressed to involve the calves, distal arms, and thighs. Clinical examination revealed symmetrical plaques on forearms and calves, featuring erythematous, hyper, and hypopigmented elements extending proximally, a positive "groove sign" and a moderate difficulty in knee joint flexion. Despite these findings, the patient was generally in good condition, without any other notable clinical signs. Initial laboratory findings showed slightly increased percentual eosinophil levels, elevated C-reactive protein (CRP), normal erythrocyte sedimentation rate (ESR), and negative antinuclear and scleroderma specific antibodies. Magnetic resonance imaging (MRI) demonstrated enhanced fascial signal and thickening while the fascia-muscle biopsy revealed marked edema and inflammatory lymphoplasmacytic infiltrate, consistent with the diagnosis of EF. The patient showed a favorable response to systemic corticosteroids. EF predominantly affects males aged 30 to 60 and is characterized by a sudden onset and unclear etiological factors. Differential diagnosis requires careful exclusion of scleroderma and other mimicking conditions. Diagnostic modalities such as skin-muscle biopsy and MRI reveal characteristic findings like inflammatory infiltrate and fascial thickening. Accurate diagnosis and differentiation from scleroderma are crucial, with early intervention involving glucocorticoids and immunosuppressive agents improving long-term outcomes.

Microbiota-Accessible Boron-Containing Compounds in Complex Regional Pain Syndrome.

The microbiota-gut-brain axis has garnered increasing attention in recent years for its role in various health conditions, including neuroinflammatory disorders like complex regional pain syndrome (CRPS). CRPS is a debilitating condition characterized by chronic neuropathic pain, and its etiology and pathophysiology remain elusive. Emerging research suggests that alterations in the gut microbiota composition and function could play a significant role in CRPS development and progression. Our paper explores the implications of microbiota in CRPS and the potential therapeutic role of boron (B). Studies have demonstrated that individuals with CRPS often exhibit dysbiosis, with imbalances in beneficial and pathogenic gut bacteria. Dysbiosis can lead to increased gut permeability and systemic inflammation, contributing to the chronic pain experienced in CRPS. B, an essential trace element, has shown promise in modulating the gut microbiome positively and exerting anti-inflammatory effects. Recent preclinical and clinical studies suggest that B supplementation may alleviate neuropathic pain and improve CRPS symptoms by restoring microbiota balance and reducing inflammation. Our review highlights the complex interplay between microbiota, inflammation, and neuropathic pain in CRPS and underscores the potential of B as a novel therapeutic approach to target the microbiota-gut-brain axis, offering hope for improved management of this challenging condition.

Time to redefine hyperuricemia? The serum uric acid cut-off level for precipitation might be lower: a pilot study.

BACKGROUND: Hyperuricemia is classically defined as serum uric acid (SUA) value higher than 6.8 mg∕dL; between hyperuricemic patients, only 15-20% will develop gout. Our first goal was to find if there is a specificity of the "snowstorm" feature on ultrasound (US) for hyperuricemia. Moreover, we aimed to determine if there is a level of SUA from which the urates tend to appear in the synovial fluid, without generating a typical clinical gouty flare. PATIENTS, MATERIALS AND METHODS: We conducted a cross-sectional, transverse study, including 108 consecutive patients that displayed a set of clinical and imaging features, such as swollen knee and US proof for knee joint effusion. RESULTS: Performing binary logistic regression, the relation between the explanatory variable (hyperechogenic spots) and the response variable (SUA) was demonstrated to be a significant one (p=0.005). The value of 0.397 for the statistical phi coefficient suggests a medium intensity association between the diagnosis of gout or asymptomatic hyperuricemia and whether the patients have hyperechogenic spots or not. We found the cut-off value for SUA equal to 4.815 mg∕dL, regardless of gender, from which, the urate starts to precipitate. Values for men tend to be higher in comparison to the ones found for women (4.95 mg∕dL vs. 3.9 mg∕dL). CONCLUSIONS: The "snowstorm" aspect of the fluid might be the result of an increased level of SUA and more than this, the cut-off level for SUA to precipitate might be lower than the fore used values.

Staphylococcus aureus-induced septic arthritis of the ankle related to malum perforans in a diabetes patient.

Septic arthritis (SA) is a less common joint pathology with potentially fatal outcome. It is considered a medical emergency, in which prompt diagnosis and differentiation of bacterial etiology is essential for appropriate management. The knee is the most prevalent site for SA (~50% of cases), followed by hip, shoulder, and elbow. Early intervention requires an accurate diagnosis and imaging techniques enable both a positive diagnosis, as well as arthrocentesis and liquid analysis, the "gold standard" criteria. We report the case of a 70-year-old patient, with history of rheumatoid arthritis (RA), diabetes mellitus (DM) and persistent left malum perforans in the last year, with development of a severe and debilitating Staphylococcus aureus-related SA of the left ankle, which posed significant therapeutic challenges. He developed a plantar lesion at the ball of the left foot, in the past one year, which was labeled as malum perforans in the setting of DM. Musculoskeletal ultrasound was the primary imaging technique used to define the location and extent of the infectious process. Cultures drawn from the tissue were positive for S. aureus. After an antibiotic course, the apparent infectious features were remitted but the long-lasting open wound failed to improve. Antibiotic therapy was initiated in accordance with culture sensibility tests but short- and long-term outcome was unfavorable with both treatment unresponsiveness and comorbidity burden posing considerable difficulties. The association and interrelation between different comorbidities (such as hypertension, diabetes, or obesity), chronic systemic inflammation (e.g., C-reactive protein level, disease activity), and RA medication is sometimes difficult to understand and to address in daily practice, and this case report highlights multiple toils encountered in a SA patient with RA on immunosuppressive therapy and complicated DM.

A Study on Dermatomyositis and the Relation to Malignancy.

The idiopathic inflammatory myopathies (IIM) are a group of heterogeneous systemic diseases which include as main subtypes: polymyositis (PM), dermatomyositis (DM) and inclusion body myositis (IBM). The key feature of IIMs is the muscle weakness, accompanied by a characteristic skin rash in DM patients. The overall risk for malignancy in IIM is higher compared to the age-and sex-matched general population. Most epidemiologic studies have included only PM and DM patients and reported consistently higher rates of malignancy in DM. Most common types of cancer in DM are adenocarcinoma of the lung, ovary or gastrointestinal tract, melanoma and non-Hodgkins lymphoma. The highest risk for malignancy is seen in the first year after DM diagnosis. Multiple disease features have been linked to the development of cancer in DM. These include: older age, male sex, skin necrosis, Gottron sign, heliotrope rash, dysphagia, low complement C4, lymphocytosis, poor response to corticosteroids and rapid disease progression. Our study included 23 patients with DM, divided into two groups based on the association of malignancy, in order to compare clinical and demographic features, laboratory markers and analyze characteristic of cancer development.

Correlations between infection patterns and eye damage in reactive arthritis.

The purpose of the study was to correlate the infectious patterns (urogenital, enteral) with ocular manifestations, such as conjunctivitis or acute anterior uveitis in reactive arthritis (ReA). The transversal, observational study was conducted in the Clinic of Rheumatology, University of Medicine and Pharmacy of Craiova, Romania, during the period 2012-2015, and included 112 patients. They were divided into three groups, as follows: Group I (52 ReA cases), Group II (30 other spondyloarthritis cases), Group III (40 osteoarthritis cases). Ophthalmologic screening was performed, together with the detection of human leukocyte antigen B27 (HLA-B27) and the infectious trigger. Within Group I, 42 (80.76%) patients presented genitourinary infections (78.57% Chlamydia trachomatis, 21.43% Ureaplasma urealyticum), 10 (19.23%) patients presented enteral infections (Yersinia enterocolitica and Salmonella). Compared with the other two groups, the patients from the first group were documented with more conjunctivitis episodes. Although anterior uveitis is more common in ankylosing spondylitis (AS), in the present study more cases were identified in Group I (11.9%) than in Group II (6.67%) and no case were recorded in Group III. Most of patients from Group I were HLA-B27 positive. The etiologic profile of ocular manifestations in ReA is dominated by genitourinary infections - C. trachomatis and U. urealyticum, respectively. Cases of recurring uveitis are associated with elevated levels of anti-immunoglobulin A (IgA).

Complex regional pain syndrome as a paraneoplastic disorder associated with metatypical basal cell carcinoma.

Metatypical basal cell carcinoma (MTBCC) is a rare form of tumor, which associates the clinical and histopathological (HP) characteristics of both basal cell carcinoma (BCC) and squamous cell carcinoma (SCC), with a 5% chance for the development of metastases. The reference diagnosis remains the HP confirmation from the lesional tissue. The current report illustrates the case of a 74-year-old patient, diagnosed with MTBCC consequently to the biopsy from the clinically malignant lesion with HP and immunohistochemical examination, currently in clinical remission following surgical treatment. The musculoskeletal symptoms represent the patient's admission reason to the Clinic of Rheumatology, where he was diagnosed with paraneoplastic type I complex regional pain syndrome (CRPS-I). The onset was six weeks prior with intense pain in the upper limb, burning sensation and nondermatomal distribution, exacerbated by lowering the position of the upper limb. The clinical evaluation revealed vasomotor disorders: color changes on the skin of the upper limb, venous turgescence on the back of the hands, and local increased temperature. Also, there were evident sudomotor modifications with hyperspiration and fluffy edema. The presence of clinical manifestations associated with the HP confirmation of MTBCC and the information provided by the imaging tests regarding the evaluation of tumor extension advocates for the diagnosis of paraneoplastic CRPS, consequently to both the primary tumor and the pulmonary metastasis. Diagnosis of CRPS-I is generally established on the basis of clinical criteria after excluding other conditions that may explain the degree of pain and the existing dysfunction. The therapist should be aware of the clinical manifestation of CRPS, as early recognition and aggressive treatment often leads to the best response.

Neuroinflammation in systemic lupus erythematosus - a review.

Neuroinflammation is a complex process that contributes to the pathogenesis of both immune mediated and neurodegenerative pathologies. Systemic lupus erythematosus (SLE) is the prototype of connective tissue diseases that can present the complete spectrum of neurological and psychiatric dysfunctions. The precise etiological diagnosis of neuropsychiatric systemic lupus erythematosus (NPSLE) is rather difficult to be established and it is still controversial the exact timing of neuropsychiatric (NPS) events: either central nervous system (CNS) is the initial target of autoimmune abnormalities, either NPS symptoms are a part of multisystem involvement. Ischemic and inflammatory mechanisms have an important input on NPSLE pathogenesis. Neuroinflammation, consequent to blood-brain barrier (BBB) damage, local and systemic production of autoantibodies, determine neuronal injury and apoptosis, further responsible for diffuse cerebral events, mostly cognitive dysfunction and psychotic disorder. Moreover, SLE complications or therapy complications can interfere and contribute to complex clinical manifestations that can be present in SLE patients. Understanding the role of each pathogenic way can provide not only an early diagnosis, but a more accurate therapeutic approach of these patients.

Behçet's disease with rapidly progressive bilateral optic neuropathy and avascular femoral neck necrosis. Literature review and management update.

Behçet's disease is a multi-systemic vasculitis of small arteriolar and venular vessels, which shows a wide range of clinical manifestations, such as oral and genital aphthosis, erythema nodosum, panuveitis, complex gastrointestinal disorders, the early onset of neurological involvement being a negative prognostic factor in evolution. We present the case of a 36-year-old patient, who was admitted in the Clinic of Rheumatology for recurrent-neglected oral aphthosis, recurrent erythema nodosum, left hip pain, reduction of visual acuity of the right eye, weight loss, profuse sweating, marked fatigability. From the personal history was retained avascular necrosis of right femoral head, with arthroplasty at this level, human leukocyte antigen-B51 (HLA-B51) positive. Ophthalmological evaluation reveals severe bilateral optic neuropathy, with confirmation of neuro-Behçet's disease (NBD) diagnosis, in a Neuro-Ophthalmological Center, based on cerebral nuclear magnetic resonance and cerebrospinal fluid analysis. Associated corticosteroid therapy with Azathioprine was initiated, with no signs of activity and progression of the disease in evolution. The case provides a necessary upgrade of the therapeutic strategies specific to the NBD pattern, emphasizing the importance of the multidisciplinary approach of a patient with complex pathology.

Left gluteal metastasis from a hepatocellular carcinoma - an unusual finding.

Hepatocellular carcinoma (HCC) is the primary malignant tumor of the liver that is directly derived from parenchymal cells. It is usually encountered in patients already suffering from a long-established liver disease that has evolved past the stage of liver cirrhosis. It is usually associated with viral liver infections, alcohol consumption or other dietary habits that lead to liver damage. Metastases are not rare and are usually found incidentally after a period of monitoring the main liver disease. We present here a rare case of HCC metastasis found in the right gluteal region, in a hepatitis C virus-infected patient also displaying lung tumor lesions. Diagnosis of both the metastasis and of the primary tumor were found during the same hospital visit, employing contrast-enhanced computed tomography, magnetic resonance imaging and ultrasound (US), with positive biopsy of the metastatic lesion, performed under US guidance. The patient received oncological treatment, with good prognosis and stable evolution during the next eight months since diagnosis.

The role of skin and muscle biopsy in the diagnosis of main connective tissue diseases.

Systemic involvement in autoimmune diseases is often unclear and organ changes are confounding, thus making it difficult to have an early accurate diagnosis. In those situations, both clinical and paraclinical findings might orientate the diagnosis, but only histological or immunohistochemistry changes might be accurate enough. The skin histological changes are relevant and sometimes might have a tremendous role in the accurate diagnosis of autoimmune rheumatic diseases, due to the correlation with the clinical systemic manifestations of the diseases and through the accessibility of biopsy. In the same time, muscle biopsy can provide important support for physicians improving diagnosis and optimizing management of connective tissue diseases.

Intramuscular hemangioma of the arm: ultrasonography and pathology features.

Hemangiomas are between the most frequent soft tissue masses and despite the vascular origin, they do not generate metastases and do not have malignant evolution. They are frequent in childhood and female sex is more frequently affected. If deeply located, these tumors are difficult to diagnose and thus an imaging method is often needed, but conventional radiology is not sufficient. If superficially located, it frequently involves the skin and subcutaneous tissues, but in the deeper layer, they are often intramuscular. Clinical findings of intramuscular hemangioma include swelling, pain and sometimes loss of muscle function.

Ultrasonography role in the evaluation of a giant cell tumor of the flexor pollicis longus tendon.

Hand tendons lesions represent a challenge for an accurate diagnosis, an optimal treatment strategy, the description of the lesion and its location being an important step. The non-invasive ultrasound evaluation was demonstrated to be an important diagnostic method in these types of lesions, especially in those situations where clinical evaluation failed to reveal the pathological changes and therefore has an important role in the adequate management.

Vascular endothelial growth factor in systemic lupus erythematosus - correlations with disease activity and nailfold capillaroscopy changes.

Our study aimed to quantify serum VEGF (vascular endothelial growth factor) and its inter-relation with the severity of microvascular damage, assessed by nailfold capillaroscopy (NC), and to establish the possible relationship with disease activity score. We included 18 patients, diagnosed with systemic lupus erythematosus (SLE) and 17 gender and age-matched control subjects. For determining serum VEGF, we used a Human VEGF Assay kit-IBL. NC was performed, according to the standard method, using a video-capillaroscope Videocap 3.0, DS Medica, by the same examiner, blinded to clinical and laboratory data. Serum VEGF registered a mean value of 68.99±71.06 pg/mL for SLE patients and 31.84±11.74 pg/mL for controls, differences statistically significant; depending on disease activity, we found a mean value of 60.11±57.74 pg/mL, for patients with moderate disease activity vs. 30.96±11.51 pg/mL for the ones with a low activity (p=0.014). We found a moderately positive correlation, statistically significant (p=0.015), between serum level of VEGF and Systemic Lupus Erythematosus Disease Activity Index (SLEDAI). Performing NC, we found changes in 88.88% of the patients; the most frequent were increased tortuosity, dilated capillaries, an increased length and a prominent subpapillary plexus. The presence of nailfold capillaroscopy changes and serum level of VEGF, correlated moderately, positive. Since serum levels of VEGF are higher in SLE patients, compared to controls, significantly different according to disease activity degree, and directly inter-related to abnormal NC patterns and a more active disease, we can include these accessible parameters in the routine evaluation, in order to better quantify the systemic damage, individualize the treatment, improve the outcome and life quality for these patients.