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Calcific aortic valve disease (CAVD) is a progressive cardiovascular disorder pathologically defined by valvular sclerosis, fibrosis, and ectopic mineralization, which constitutes a substantial and growing public health burden. Currently, surgical intervention represents the sole effective treatment, underscoring a critical unmet need for novel pharmacological strategies that can halt disease progression or provide early therapeutic intervention. Extensive research has established that the pathogenesis of CAVD is driven by a complex interplay of multiple mechanisms including inflammatory responses, oxidative stress, and metabolic dysregulation which are intricately modulated by epigenetic regulation, post-transcriptional modifications, and protein post-translational modifications. In recent years, the field of epigenetics has garnered considerable attention, particularly for its pivotal role in the pathogenesis of oncological and cardiovascular diseases and the subsequent development of targeted therapeutic strategies. Consequently, numerous investigations have been dedicated to elucidating the involvement of epigenetic mechanisms in CAVD, encompassing DNA methylation, histone modifications (including methylation and acetylation), and RNA methylation, with a pronounced emphasis on the regulatory functions of non-coding RNAs. This review synthesizes recent advances in our understanding of epigenetic mechanisms underlying CAVD, with a specific focus on the role of RNA N6-methyladenosine (m6A) methylation, and highlights the pivotal significance of epigenetic modulation in critical biological processes and CAVD pathogenesis. Collectively, these findings offer valuable mechanistic insights and may illuminate novel paths toward the clinical translation of epigenetically targeted therapies for CAVD.

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The prevalence of calcific mitral stenosis (MS), which is associated with mitral annular calcification (MAC), has been increasing, particularly in aging populations such as in Japan. Severe MAC interferes with the normal diastolic relaxation of the mitral annulus, and calcification can extend onto the mitral leaflets with reduced leaflet mobility, causing MS. As MAC frequently coexists with aortic stenosis, aortic stenosis is a strong risk factor for calcific MS among patients with MAC. The advent and expansion of transcatheter aortic valve implantation revealed attendant challenges of calcific MS. The diagnosis and severity assessment of calcific MS differ from rheumatic MS. Further, the combination of aortic and mitral stenoses complicates the hemodynamic assessment. Calcific MS is frequently observed in elderly patients with multiple comorbidities; therefore the prognosis of calcific MS is influenced by these comorbidities as well as the patient's general condition. Surgical mitral valve replacement is considered an optimal treatment for MS. However, severe MAC poses significant challenges for surgeons, often requiring debridement, decalcification, and annular reconstruction, which lead to an increased surgical risk. Because perioperative mortality can be high in these patients undergoing high-risk cardiac surgery, medical therapy is preferred, and less invasive surgical procedures or catheter interventions are considered. This review integrates the latest insights into the pathophysiology, diagnosis, prognosis, and treatment options of calcific MS, highlighting its clinical challenges.

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Calcinosis is a severe complication of adult and juvenile dermatomyositis (DM/JDM), characterized by the deposition of calcium in the skin, muscles, and soft tissues, leading to significant morbidity. This review explores the pathogenesis of DM/JDM-associated calcinosis, highlighting the roles of chronic inflammation, neutrophil extracellular traps, proinflammatory cytokines, and mitochondrial stress. Current therapeutic approaches including anti-inflammatory agents and emerging treatments such as chimeric antigen receptor T (CAR-T) cells are reviewed. Additional therapies targeting calcium metabolism are also examined. Despite the variety of treatments, there is a need for controlled trials with validated outcomes to understand the efficacy and safety of these therapies.

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Pulmonary alveolar microlithiasis is an ultrarare autosomal recessive lung disease caused by loss of a key pulmonary epithelial sodium phosphate cotransporter resulting in elevation of luminal phosphate and accumulation of calcium phosphate crystals in alveolar spaces. The disease is often discovered incidentally on routine chest films in asymptomatic individuals or through screening of families with a known history of pulmonary alveolar microlithiasis (PAM) and tends to progress slowly, often culminating in respiratory failure in late middle age. Current management is limited to supportive measures, with lung transplantation in end stage disease. Recent advances in the pathogenesis of PAM have suggested therapeutic approaches.

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OBJECTIVE: Rotator cuff calcific tendinopathy is a leading cause of nontraumatic shoulder pain, frequently leading to articular and functional impairments, depicting an adhesive capsulitis-like clinical presentation. To date, there is a lack of evidence on the impact of conservative approaches, and no gold standard has been established for managing rotator cuff calcific tendinopathy. This systematic review aimed to identify the most effective conservative approach for reducing pain and improving function in rotator cuff calcific tendinopathy patients. METHODS: PubMed, Scopus, and Cochrane Library databases were systematically searched from their inception until January 2, 2025, for English-language randomized clinical trials including adults affected by rotator cuff calcific tendinopathy undergoing conservative treatment. Data extraction was performed independently by two reviewers using a customized data extraction form, with consensus reached by a third reviewer. A network meta-analysis was subsequently carried out to compare the efficacy of different interventions. The risk of bias within the included randomized clinical trials was assessed using Version 2 of the Cochrane risk-of-bias tool for randomized trials. The study has been registered with PROSPERO, registration number CRD420250650833. RESULTS: Nineteen articles were included. This study identified 1160 subjects affected by rotator cuff calcific tendinopathy. A pairwise comparison through a network meta-analysis indicated that platelet-rich plasma exhibited the highest probability (85%) of improving shoulder function, followed by disodium ethylenediamine tetra-acetic acid at 75%, aspiration techniques at 65%, and extracorporeal shockwave therapy at 57%. Regarding pain reduction, disodium ethylenediamine tetra-acetic acid showed the highest probability (66%), followed by kinesiotaping and needle aspiration, both at 61%. CONCLUSION: This systematic review and network meta-analysis identified several interventional techniques, including platelet-rich plasma and disodium ethylenediamine tetra-acetic acid injections, extracorporeal shockwave therapy, and needle aspiration, as more effective strategies for reducing pain and improving function in subjects affected by rotator cuff calcific tendinopathy. LEVEL OF EVIDENCE: I (systematic review of Level-I randomized controlled studies).

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OBJECTIVE: Calcific aortic valve disease advances via increased fibrosis and calcification deposition, leading to progressive narrowing of the outflow tract, left ventricular hypertrophy and cardiac failure. Currently, surgical repair and replacement are the only strategies for intervention. While transcatheter aortic valve replacement offers a less invasive alternative to surgical intervention, implanted bioprosthetic valves have limited durability. Non-invasive ultrasound therapy was shown to not damage normal porcine aortic valve, and successfully reduced stenosis of calcified bioprosthetic valves in vivo, as well as decrease stenosis of native severely calcified aortic stenosis in a first-in-human study. However, critical questions regarding the effect of ultrasound therapy on valvular cells remain. In this study, we aimed to optimize pulsed ultrasound cavitation (PUC) treatment for cell culture studies and evaluate the acute and persistent effects on human valvular interstitial cells (hVICs) in calcifying conditions. METHODS: We utilized viability, metabolism and calcification assays as well as mass spectrometry-based proteomics to holistically characterize the effects of PUC treatment in hVICs. RESULTS: hVICs viability and metabolism were not significantly altered as a function of PUC treatment at short- (48 hour) or long-term (21 day) time points. Furthermore, PUC treatment did not increase hVICs calcification in vitro. Proteomic profiling of hVICs showed that PUC treatment had limited persistent changes to protein profiles compared with the acute effects of PUC treatment. CONCLUSION: These studies suggest that ultrasound therapy, currently limited to very severe aortic stenosis, could be of interest at an earlier stage of the disease.

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BACKGROUND: Cardiac calcification has multiple etiologies, including previous myocardial infarction, sepsis, and myocarditis. An imbalance between plasma calcium and phosphorus levels can also lead to cardiac calcification. Patients with pre-existing conditions such as rheumatic heart disease or those who have undergone mitral valve replacement (MVR) are at risk of developing left atrium (LA) calcification. Complications may include pulmonary hypertension and heart failure. The objective of this study is to summarize left atrial calcification, its causes, complications, and therapeutic guidelines. METHODS: This study reviewed relevant literature from Google Scholar and PubMed databases until October 2024, including case series, observational studies, and randomized controlled trials (RCTs). The American Psychological Association (APA) seventh edition guidelines were followed for data presentation. RESULTS: Coconut LA can arise from various diseases, including rheumatic heart disease, congenital pulmonic valve disease, and MVR. Pathological studies indicate that atrial calcification results from collagen-rich matrices, macrophages, and lymphocytes. Radiological imaging plays a crucial role in assessing disease severity and localization. Treatment options include surgical intervention or resynchronization. CONCLUSION: In summary, calcification can affect nearly every part of the heart, with diverse underlying reasons and etiologies such as rheumatic heart disease and congenital pulmonic valve disease. Both surgical and resynchronization methods are available for patient management. However, further research is necessary to enhance our understanding of the coconut LA (calcification of the LA).

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Calcific aortic valve stenosis (CAVS) is the most frequent valve disorder in adults, with a steadily increasing incidence with age. Currently, no effective treatments are available to prevent or delay disease progression. In addition to progressive calcification, there is increasing recognition of the underlying roles of oxidative stress, chronic inflammation, lipoprotein deposition, and induction of osteogenic signaling in driving progression of CAVS. Although traditional markers of CAVS progression, such as increased valve gradients and reduced areas, are currently used to guide clinical decisions regarding valve replacement, these measures may not capture progression of early, potentially modifiable disease. Hence, there is a need for more sensitive markers, such as aortic valve calcification, to monitor disease progression in CAVS. This JACC State-of-the-Art Review provides a comprehensive discussion of the pathogenesis and progression of CAVS and emphasizes the unmet need for innovative medical treatments. Additionally, it explores emerging therapeutic approaches, advanced methods for evaluating disease progression, and cutting-edge techniques to measure therapeutic efficacy.

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BACKGROUND: Ectopic calcification, especially in soft tissues such as subcutaneous adipose tissue, is a rare and serious complication in chronic kidney disease (CKD) patients. It is commonly associated with cardiovascular morbidity and mortality. This case report highlights the occurrence of multiple ectopic calcifications in a patient with end-stage renal disease (ESRD) on peritoneal dialysis, emphasizing the role of inflammatory cytokines in the pathogenesis of this condition. Early and appropriate intervention can facilitate partial reversibility, highlighting the importance of regular follow-ups and the optimization of prescriptions, particularly in ensuring intensified high-quality, goal-oriented dialysis. CASE PRESENTATION: A 68-year-old female patient with diabetic nephropathy had been on maintenance peritoneal dialysis for four years. She presented with multiple subcutaneous nodules, particularly in the abdomen and lower limbs, for over a month. Ultrasound and non-contrast CT imaging revealed hyperechoic lesions and calcifications in the subcutaneous tissue, along with vascular and kidney calcifications. Laboratory results indicated inadequate dialysis, hypocalcemia, hyperphosphatemia, and significantly elevated serum parathyroid hormone (PTH) and inflammatory cytokines, including IL-6. The biopsy of the subcutaneous nodule from the lower abdomen revealed calcification and inflammation, accompanied by pronounced IL-6 expression. Treatment included intensified automated peritoneal dialysis (APD) combined with icodextrin peritoneal dialysis solution for long-term retention in the abdomen, cinacalcet, phosphate binders, calcitriol, and nutritional support. After one month, the patient's condition showed significant improvement, with reduced calcification confirmed by follow-up ultrasound. CONCLUSIONS: This case underscores the rarity of subcutaneous adipose tissue calcification in ESRD patients and highlights the crucial role of inflammatory factors, particularly IL-6, in the development of ectopic calcifications. Early, targeted interventions, especially high-quality, goal-directed dialysis, can significantly improve outcomes, illustrating the importance of regular monitoring and tailored treatment in preventing and managing calcification in CKD patients. CLINICAL TRIAL NUMBER: Not applicable.

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Rheumatic mitral stenosis (MS) with significant calcification presents challenges for both surgical and transcatheter interventions. Percutaneous balloon mitral valvuloplasty (PBMV) is often limited in these cases due to valve rigidity, increasing the risk of mitral regurgitation (MR). Transcatheter mitral valve lithotripsy (TMVL) is an emerging technique that uses sonic waves to disrupt calcification, enhancing valve pliability and PBMV outcomes. A 39-year-old woman, 18 weeks pregnant, with a history of rheumatic heart disease and prior surgical mitral commissurotomy, presented with palpitations, fatigue, and exertional breathlessness. She had severe MS (mitral valve area 0.8 cm²) and severe tricuspid regurgitation, with atrial fibrillation and rapid ventricular response. Due to a high Wilkins score, PBMV was attempted with a 28 mm Inoue balloon inflated to 28 mm, but was suboptimal due to significant valve rigidity. Adjunctive TMVL improved valve pliability, successfully reducing the mitral gradient and increasing valve area without worsening MR. The patient continued her pregnancy without complications. This case highlights TMVL as a promising adjunct to PBMV in severe calcified MS, particularly in high-risk patients such as pregnant women. Further studies are needed to validate its efficacy and long-term outcomes.

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BACKGROUND: Calciphylaxis, also termed calcific uremic arteriolopathy (CUA) in patients with end-stage kidney disease (ESKD), is a rare and fatal condition characterized by cutaneous ischemic necrosis. METHODS: Three patients with calciphylaxis and metastatic pulmonary calcification (MPC) were treated with human amnion-derived mesenchymal stem cells (hAMSCs). Effects were evaluated using the Visual Analogue Scale (VAS), modified Bates-Jensen Wound Assessment Tool for CUA (BWAT-CUA), wound quality of life questionnaire (Wound-QoL), and histological analysis. MPC was assessed by high-resolution CT (HRCT) and 99ᵐTc-methylene diphosphonate (99ᵐTc-MDP) bone scans.99ᵐTc-labeled macroaggregated albumin (99ᵐTc-MAA) pulmonary perfusion imaging was conducted for the first time in patients with MPC. RESULTS: Three patients exhibited wound healing and improvement in skin symptoms. Two months before CUA, asymptomatic MPC was detected in Patient 1, who was treated with hAMSCs for 15 months. The condition progressed to chest pain and dyspnea. HRCT and 99ᵐTc-MDP bone scans showed worsening calcification, particularly in the upper and mid-thoracic lobes.99ᵐTc-MAA pulmonary perfusion imaging revealed impaired or absent blood perfusion in the areas of metastatic calcification. Patient 1 died from respiratory failure. Patients 2 and 3 had asymptomatic MPC at calciphylaxis diagnosis. After 2 months of treatment, Patient 2, showed no significant imaging improvement and passed away 6 months after discontinuing hAMSC treatment. Patient 3 has shown no significant progression of pulmonary lesions and continues hAMSC therapy. CONCLUSION: We reported personalized early, noninvasive diagnosis and regenerative treatments for calciphylaxis patients with MPC. Although the current hAMSC treatment regimen is effective for skin lesions, its impact on MPC requires further investigation.

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Calcific aortic valve disease (CAVD) is one of the most prevalent heart valve diseases and is characterized by progressive stiffening and calcification of the aortic valve. For decades, CAVD has been treated with surgical intervention. In recent years, some progress has been made in understanding the pathogenesis of CAVD and the exploration of novel therapeutic strategies, leading to the identification of potential therapeutic targets and innovative treatment approaches. This review systematically outlines the pathophysiological advances in CAVD over the past 5 years, proposing a 3-stage model for disease progression: inflammatory, fibrotic, and calcification stages. In addition, recent clinical trials investigating pharmacological therapies, such as those targeting lipid metabolism, vitamin K pathways, and calcium-phosphorus balance, are summarized and discussed. These developments hold promise for improving patient outcomes and revolutionizing the management of CAVD.

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Retropharyngeal calcific tendinitis is an inflammation of the tendon of the long neck muscle secondary to the deposition of calcium hydroxyapatite crystals. The condition is rarely described in the literature, and cases are frequently not identified because they resolve spontaneously. Because of the nonspecific symptoms, other more serious conditions are often suggested first, such as retropharyngeal abscess or meningitis, leading to invasive and unnecessary interventions and treatments that could be avoided by a better understanding of this pathology by primary care physicians.

La tendinite calcifiante rétropharyngée est une inflammation du tendon du muscle long du cou secondaire à un dépôt de cristaux d'hydroxyapatite de calcium. Il s'agit d'une affection peu décrite dans la littérature, les cas n'étant fréquemment pas identifiés car de résolution spontanée. À cause de symptômes non spécifiques, d'autres affections plus graves sont souvent évoquées en premier lieu comme l'abcès rétropharyngé ou la méningite, ce qui entraîne la réalisation d'interventions et de traitements invasifs et inutiles susceptibles d'être évités par une meilleure connaissance de cette pathologie par les médecins de premier recours.

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Brain calcification is often detected incidentally, but basal ganglia calcification has a wide differential diagnosis, including genetic and acquired causes. Primary familial brain calcification (PFBC) (formerly 'Fahr's disease') refers to neurological disorders characterised by bilateral, symmetrical deposition of calcium-hydroxyapatite crystals in the basal ganglia and other encephalic regions, with a presumed genetic basis. Its clinical picture encompasses motor, cognitive and psychiatric manifestations in various combinations. Seven genes have been linked to PFBC since 2012, with either autosomal dominant (SLC20A2, PDGFRB, PDGFB and XPR1) or recessive (MYORG, JAM2 and NAA60) mode of inheritance. Mendelian gene discovery has provided critical insights into the pathogenesis of PFBC. Dyshomeostasis of inorganic phosphate, impaired endothelial functions and disrupted blood-brain barrier integrity has been identified as converging pathomechanisms, which could highlight the targets of potential disease-modifying treatments. We provide a state-of-the-art overview on phenotypic features, diagnosis, aetiopathogenesis and management of PFBC.

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RATIONALE: Calcific tendinitis frequently occurs in the shoulder, and while it may also occur in other joints, such as the hip, knee, wrist, and finger joints, its occurrence in the elbow joint is relatively rare. Therefore, it is susceptible to delayed diagnosis or misdiagnosis at the initial occurrence. Appropriate imaging and treatment should be considered immediately when suspecting calcific tendonitis. PATIENT CONCERNS: We detailed a young female who presented with progressive right elbow pain for the past 3 years with no past injury to the elbow. She had undergone conservative management for 2 years but was unsuccessful. Her pain became more intense 1 week prior, to the extent that she could not use her right upper limb to perform daily activities, and her sleep was severely disturbed. Therefore, she needed a possible therapeutic relief. DIAGNOSIS: Clinical findings included elbow stiffness and tenderness of the lateral epicondyle of the humerus, and Cozen assessment was positive. Computed tomography showed a high-density macular focus and smooth edge of the lateral condyle of the right humerus and dorsal side of the ulnar olecranon. The plain radiograph showed patches in the right lateral condyle of the humerus and the dorsal part of the olecranon, indicating calcification of common extensor tendinosis. INTERVENTIONS: Initially, conventional care was given to the patient with analgesics, physical therapy, or a resting regimen to decrease the pain and reduce the tedious load on the extensor tendon. Measures such as pain score, range of motion, and follow-up imaging after 8 weeks began until 2 years. However, her pain did not improve. Therefore, she was advised for surgical therapy and subsequently underwent surgical exploration of the elbow under general anesthesia. Histopathological examination of the excised tissue revealed fibrous ligament tissue with calcifications. OUTCOMES: At the 6-month follow-up, the postoperative plain radiograph showed complete removal of the heterotopic bony growth from the lateral epicondylar area with no recurrence of the heterotopic bone formation around the elbow joint. She had no pain with full elbow function and a full range of elbow extension and flexion (visual analog scale score of 0/10). LESSONS: Calcific tendonitis of the elbow is uncommon; hence, its diagnosis and treatment may be late due to its scarcity. Therefore, appropriate imaging and treatment should be considered immediately when calcified tendonitis is suspected. A literature review is also necessary since it is not a daily condition.

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BACKGROUND: Calcific rotator cuff (RTC) tendinopathy is a self-limited, atraumatic cause of shoulder pain. Ultrasound-guided percutaneous needling and aspiration (USPNA) can be used when other nonoperative treatments fail. OBJECTIVE: To determine if there is an association between morphology of RTC calcifications on imaging and the ability to aspirate calcifications. DESIGN: Case series. SETTING: Tertiary orthopedic institution. PATIENTS: A total of 272 patients who underwent USPNA for symptomatic calcific RTC tendinopathy and had available imaging and clinical data. INTERVENTIONS: Imaging was categorized using Gärtner-Heyer (x-ray), Farin-Jaroma (ultrasound), and Chiou (ultrasound) morphological classifications. MAIN OUTCOME MEASURES: Percentages of successful and unsuccessful aspirations were calculated. RESULTS: There were 292 USPNA cases; 170 (58.2%) aspirations were successful. Inter-rater reliability was almost-perfect for Farin-Jaroma and Chiou classifications (kappa: 0.92-1.00; p < .001) and fair-to-moderate for Gärtner-Heyer classifications (kappa: 0.38-0.74; p < .001). When applying the Gärtner-Heyer classification, 92/134 (68.7%) type I, 65/117 (55.6%) type II, and 10/35 (28.6%) type III calcifications were successfully aspirated (p < .001). Six calcifications could not be classified. No statistical difference was appreciated when using Farin-Jaroma (p = .939) or Chiou (p = .524) classifications. The mean calcification size for successful aspirations was significantly larger than that for unsuccessful aspirations (16.7 ± 6.4 mm vs. 13.4 ± 7.5 mm; p < .001), although there was no difference in ability to aspirate single versus multiple calcifications. The regression analysis showed an association between increasing calcification size and greater odds of aspiration success (10% increase; p < .001). Compared with type I Gärtner-Heyer classifications, type II and type III were associated with decreased odds of aspiration success (64% and 91% decreases, respectively; p ≤ .003). CONCLUSIONS: Gärtner-Heyer classification was associated with the ability to successfully aspirate calcifications; type I calcifications were most frequently aspirated. Larger calcifications were also more frequently aspirated successfully. These factors may improve patient selection for USPNA. Further studies are needed to explore whether imaging classifications of RTC calcifications are correlated with patient-reported outcomes following USPNA.

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PURPOSE: Aortic valve stenosis (AVS) is the most common valvular disease in developed countries. Surgical or transcatheter bioprosthetic aortic valve (AV) replacement is the standard treatment for severe AVS. However, bioprostheses are prone to structural degeneration. Hence, in terms of lifetime management, there is a need for therapies that can postpone AV replacement. With the aim of fragmenting calcifications and restoring AV leaflets flexibility, a new transcatheter debridement device (TDD) exploiting ultrasound is under development. We performed an ex-vivo study on human hearts to quantify how TDD treatment affects stenotic AVs hemodynamic. Additionally, a qualitative histological analysis was performed to assess TDD's impact on AV leaflets. METHODS: Three human hearts affected by AVS were characterized pre- and post-treatment in an ex-vivo beating heart simulator. To replicate physiological flowrates, a pulsatile pump was connected to the left ventricle, while a systemic impedance simulator connected to the aortic root and a reservoir connected to the left atrium closed the hydraulic circuit. Transvalvular pressure drop (ΔPsys), backflow volume, and effective orifice area (EOA) were evaluated. For histological analysis, AV leaflets sections were stained with Haematoxylin/Eosin and AlizarineRedS to highlight calcifications. RESULTS: The treatment induced a reduction in ΔPsys in all tested samples, improving EOA, but caused an increase in backflow volume. Moreover, histology suggested AV leaflets integrity. CONCLUSIONS: The TDD procedure improved AV fluid-dynamics during systole in all tested samples, without evidence of damage to tissues. This suggests TDD could be a promising option to postpone AV replacement for patients with AVS.

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A 76-year-old female with hypertension and hyperlipidemia was evaluated for incidental left ventricular calcification. Despite recent exertional chest pain, she was largely asymptomatic. Cardiac imaging revealed extensive endomyocardial calcifications without significant coronary artery stenosis. Laboratory tests excluded hypercalcemia, hyperparathyroidism, and sarcoidosis, leading to a diagnosis of idiopathic calcific cardiomyopathy. This case highlights the importance of comprehensive cardiac imaging in detecting subtle abnormalities, even in asymptomatic patients from non-tropical regions. It emphasizes considering both metastatic and dystrophic causes of endomyocardial calcification, regardless of geographical location. Given the patient's asymptomatic status and the condition's benign nature, a conservative management approach with regular monitoring was adopted. This case contributes to the limited literature on incidental endomyocardial calcification and may inform future strategies for similar presentations.

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Little has been written about the challenges in wound healing presented by rare cases of dermatomyositis (DM) complicated by glucocorticoid use. The authors explore the clinical presentation of a 60-year-old female patient with end-stage DM, chronic steroid use and delayed wound healing, requiring surgical debridement of wounds and extensive calcification removal. Her atypical presentation-lacking some of the characteristic dermal and antibody findings-is described, while also highlighting calcification and wound trials that complicated management. The underlying pathophysiology of effects on capillary networks is discussed, as well as the effectiveness of various treatment modalities, including steroids, antimetabolites and biologics, some of which were used. The report concludes with opportunities for future study on the disease's complex mechanisms.