Elevated Lp(a): Guidance for Identifying and Managing Patients.

Lipoprotein(a) (Lp(a)) is a unique low-density lipoprotein-like lipoprotein that is considered an independent and causal risk factor for atherosclerotic cardiovascular disease (ASCVD) and calcific aortic valve stenosis. The Lp(a) molecule also contains apolipoprotein A and apolipoprotein B, which collectively promote atherosclerosis, thrombosis, and inflammation. Lp(a) is highly genetic and minimally responsive to nonpharmacological measures. Lp(a) serum levels ≥125 nmol/L are associated with increased ASCVD risk, but this threshold has not been accepted universally. Elevated Lp(a) is the most common genetic dyslipidemia affecting approximately 20% of the general population. Certain currently available lipid-lowering drugs, including the proprotein convertase subtilisin/kexin type 9 therapies, produce moderate reductions in Lp(a); however, none are indicated for the treatment of elevated Lp(a). There are currently four investigational RNA-based therapeutic agents that reduce Lp(a) by 70% to 100%. Two of these agents are being evaluated for ASCVD risk reduction in adequately powered outcomes trials, with results expected in 2 to 3 years. Until such therapies become available and demonstrate favorable clinical outcomes, strategies for elevated Lp(a) primarily involve early and intensive ASCVD risk factor management.

Efficacy of intravascular lithotripsy (IVL) in coronary stenosis with severe calcification: A multicenter systematic review and meta-analysis.

BACKGROUND: With heavily calcified coronary and peripheral artery lesions, lesion preparation is crucial before stent placement to avoid underexpansion, associated with stent thrombosis or restenosis and patency failure in the long-term. Intravascular lithotripsy (IVL) technology disrupts superficial and deep calcium by using localized pulsative sonic pressure waves, making it to a promising tool for patients with severe calcification in coronary bed. AIMS: The aim of the study is to systematically review and summarize available data regarding the safety and efficacy of IVL for lesion preparation in severely calcified coronary arteries before stenting. METHODS: This study was conducted according to the PRISMA guidelines. We systematically searched PubMed, SCOPUS, and Cochrane databases from their inception to February 23, 2023, for studies assessing the characteristics and outcomes of patients undergoing IVL before stent implantation. The diameter of the vessel lumen before and after IVL, as well as stent implantation, were analyzed. The occurrence of major adverse cardiovascular events (MACE) was assessed using a random-effects model. RESULTS: This meta-analysis comprised 38 studies including 2977 patients with heavily calcified coronary lesions. The mean age was 72.2 ± 9.1 years, with an overall IVL clinical success of 93% (95% confidence interval [CI]: 91%-95%, I2 = 0%) and procedural success rate of 97% (95% CI: 95%-98%, I2 = 73.7%), while the in-hospital and 30-days incidence of MACE, myocardial infarction (MI), and death were 8% (95% CI: 6%-11%, I2 = 84.5%), 5% (95% CI: 2%-8%, I2 = 85.6%), and 2% (95% CI: 1%-3%, I2 = 69.3%), respectively. There was a significant increase in the vessel diameter (standardized mean difference [SMD]: 2.47, 95% CI: 1.77-3.17, I2 = 96%) and a decrease in diameter stenosis (SMD: -3.44, 95% CI: -4.36 to -2.52, I2 = 97.5%) immediately after IVL application, while it was observed further reduction in diameter stenosis (SMD: -6.57, 95% CI: -7.43 to -5.72, I2 = 95.8%) and increase in the vessel diameter (SMD: 4.37, 95% CI: 3.63-5.12, I2 = 96.7%) and the calculated lumen area (SMD: 3.23, 95% CI: 2.10-4.37, I2 = 98%), after stent implantation. The mean acute luminal gain following IVL and stent implantation was estimated to be 1.27 ± 0.6 and 1.94 ± 1.1 mm, respectively. Periprocedural complications were rare, with just a few cases of perforations, dissection, or no-reflow phenomena recorded. CONCLUSIONS: IVL seems to be a safe and effective strategy for lesion preparation in severely calcified lesions before stent implantation in coronary arteries. Future prospective studies are now warranted to compare IVL to other lesion preparation strategies.

A rare case of intervertebral disc calcification combined with ossification of the posterior longitudinal ligament in a child: a case report and literature review.

BACKGROUND: Intervertebral disc calcification (IDC) combined with calcification in children has been sporadically reported, while ossification of the posterior longitudinal ligament (OPLL) in the cervical spine in pediatric patients is exceedingly rare. The aim of this study is to investigate the potential prognosis and outcomes associated with this condition. CASE PRESENTATION: We present an unusual case involving a 10-year-old Chinese child diagnosed with calcified cervical disc herniation and ossification of the posterior longitudinal ligament. Conservative treatment measures were implemented, and at the 1-month and 6-month follow-up, the patient's pain exhibited significant improvement. Subsequent cervical MRI and CT scans revealed the complete disappearance of OPLL and substantial absorption of the calcified disc. During the three-month follow-up, CT demonstrated slight residual disc calcification, however, the patient remained asymptomatic with no discernible limitation in cervical motion. CONCLUSIONS: We conducted a comprehensive review of several cases presenting with the same diagnosis. It is noteworthy that IDC combined with OPLL in children constitutes a rare clinical entity. Despite imaging indications of potential spinal canal occupation, the majority of such cases demonstrate complete absorption following conservative treatment, with OPLL exhibiting a faster absorption rate than calcified discs.

Calcinosis in Juvenile Dermatomyositis-Epidemiology, Pathogenesis, Clinical Features, and Treatment: A Systematic Review.

PURPOSE OF REVIEW: We performed a systematic review of the literature on the epidemiology, pathogenesis, clinical and laboratory characterization, and treatment of calcinosis in patients with juvenile dermatomyositis (JDM). A qualitative systematic review was conducted from January 1975 to April 2023 according to the PRISMA protocol using three electronic databases: PubMed, Web of Science, and Scopus. Studies were analyzed based on the following eligibility criteria: at least one combination of the terms described in the search strategy appeared in the title, written in English, Portuguese, or Spanish, and addressed the epidemiology, pathogenesis, diagnosis, and treatment of calcinosis in juvenile dermatomyositis. Systematic or scoping reviews, letters, clinical images, book chapters, abstracts, inflammatory myopathy in other connective tissue diseases, idiopathic inflammatory myopathies in adults, and purely qualitative studies were excluded. RECENT FINDINGS: Seventy-five studies were included. According to the literature, calcinosis is common in women, around five years old, with three years of disease in association with osteoarticular, cutaneous, pulmonary manifestations, and fever. The pathogenesis is still unknown, but the participation of interleukin 1 and 6, tumor necrosis factor alpha, and innate immunity dysregulation seem to be involved. Common autoantibodies are anti-NXP-2, anti-MDA-5, and anti-Mi-2, and their treatment remains controversial. Prospective, randomized, controlled studies are needed to evaluate treatment protocols and map the natural history of this serious complication. Calcinosis seems to be more common in White female children with muscle weakness, fever, arthritis, severe pulmonary, and skin involvement with anti-NXP-2, anti-MDA-5, and anti-Mi-2 autoantibodies. The multitargets and aggressive treatment is recommended.

Intravascular lithotripsy: A novel option for severe calcification of coronary artery.

BACKGROUND: Severe coronary artery calcification is associated with low success rate of interventional operation, perioperative adverse cardiac events, and poor prognosis, which is a major problem faced by operators. The existing therapy methods all have inherent limitations, such as unsatisfactory balloon crossability, inadequate balloon dilation, and so on. The emergence of intravascular lithotripsy (IVL) has brought the dawn of the treatment of calcified lesions by using unfocused acoustic pressure waves to fracture calcification in situ. And IVL is the only technology capable of targeting deep calcification. HYPOTHESIS: IVL may have great clinical application values and potential prospects. METHOD: Based on the existing clinical evidence of IVL and traditional treatment ways, this review discusses the safety and efficacy of IVL. Combined with clinical practice, the precautions and coping strategies of IVL are analyzed. And the review improves the management algorithm of coronary calcification. RESULTS: IVL has extremely high safety and effectiveness for severe coronary calcification compared with other ways, and structural improvements of IVL will further expand its value. CONCLUSIONS: The emergence of IVL could set off a revolution in the treatment of coronary artery calcification.

Effectiveness of extracorporeal shockwave therapy in treatment of upper and lower limb tendinopathies: A systematic review and meta-analysis.

BACKGROUND: Tendinopathy is caused by repetitive motion, excessive stress on the tendon, overstretch, and motion past the critical angle of rotation or translation. There are various treatment options available for tendinitis, including those affecting both the upper and lower limbs and those of calcific and non-calcific types. HYPOTHESIS/PURPOSE: Few reviews have analyzed the efficacy of Extracorporeal Shockwave Therapy (ESWT) in treating upper and lower limb tendinopathies while considering calcific and non-calcific-type tendinitis. This paper provides a review and meta-analysis on Randomized Controlled Trials (RCTs), which compared results from a group undergoing ESWT treatment to another group under different treatments. STUDY DESIGN: Systematic review and meta-analysis. METHODS: A systematic search was conducted on PubMed, Web of Science, Embase, the Cochrane Central Register of Control Trials (CENTRAL), and Physiotherapy Evidence Database (PEDro) databases for articles published up to January 2022. The reference lists of identified articles were further scanned. Twenty-two studies were included in the meta-analysis. RESULTS: Meta-analysis showed no difference in pain reduction in ESWT when compared with a comparison group at 4 (p = 0.26) and 12 weeks (p = 0.33). There were no differences in DASH scores at 12 months between the two groups (p = 0.32). CONCLUSION: Extracorporeal Shockwave Therapy (ESWT) is effective when used to treat lower limb and calcific tendinitis. It does not yield any better results than other treatment options in managing upper limb tendinopathies and non-calcific tendinopathies.

Balloon-expandable transcatheter heart valves for treatment of aortic valve stenosis in patients with large aortic annuli: Evaluation of deployment balloon overfilling strategies.

OBJECTIVES: Transcatheter aortic valve implantation (TAVI) using balloon-expandable (BE) transcatheter heart valves (THV) in aortic annuli above 29 mm includes particular procedural steps, mainly involving overfilling of the deployment balloon. Data on overfilling strategies in clinical daily practice is scarce. We herein aimed for a retrospective description of utilized overfilling strategies in those patients. METHODS: Between January 2016 and December 2022, 45 patients (100% male, 76.9 ± 6.1 years) received TAVI in aortic annuli above 29 mm using a BE THV. Overfilling volumina of the deployment balloon were left to operators' discretion. Clinical and multislice computed tomography data were retrospectively collected. Clinical endpoints were adjudicated in accordance with the updated standardized VARC-3 definitions. RESULTS: Profound overfilling (+4/5 mL) was used in patients with a mild calcium burden (˂750 mm³) even in aortic annuli of 29.0-30.0 mm. Nominal/slight overfilling (+1 mL) was used in aortic annuli up to 32.5 mm but an intermediate to severe calcific burden (>750-3200 mm³). Accordingly, a low calcification group (˂750 mm³, n = 17) compared to a significant calcification group (≥750 mm³, n = 28), presented with higher overfilling volumina (2.1 ± 1.4 vs. 0.8 ± 1.0; p ˂ 0.001), although aortic annulus diameter was not different (29.8 ± 0.8 vs. 29.9 ± 0.9 mm; p = 0.7). All-cause 30-day mortality was 0%. Device success was 97.8%. Transvalvular mean pressure gradient at discharge was 9.5 ± 3.6 mmHg. No case of PVL >mild was documented. CONCLUSION: Extent of overfilling of the deployment balloon largely depends on calcification burden in addition to aortic annulus diameter with significant and profound overfilling particularly in patients with a calcification burden of the aortic valve complex ˂750 mm³.

Bibliometric analysis of extracorporeal shock wave therapy for tendinopathy.

BACKGROUND: Extracorporeal shock wave therapy (ESWT) is a mature, conservative treatment modality for tendinopathy. Although many relevant studies have been conducted, systematic bibliometric studies are lacking. This study aimed to identify trends and hotspots in the treatment of tendinopathy using ESWT. METHODS: A literature search was conducted on ESWT for tendinopathy using the Web of Science Core Collection with a search period of 2002 to 2022. Of 559 identified studies, 276 met the inclusion criteria and were analyzed using CiteSpace software. RESULTS: The results showed that from 2002 to 2022, the publication rate of literature on ESWT for tendinopathy was generally increasing. Research hotspots, such as tendinopathy and calcific rotator cuff deposits, began earlier but continued to receive scholarly attention. Research on animal models and molecular mechanisms has progressed slowly in this field. The combined or comparative effectiveness of injectable and supplement-based treatments with ESWT is a popular research topic. CONCLUSION: Pain management in patients with tendinopathy has received considerable attention. Simultaneously, more clinical indicators of energy levels and pulse parameters during ESWT are needed to provide more scientific and accurate treatment for patients.

Modified leopard-crawl technique using intravascular lithotripsy balloon in calcified coronary lesions.

Intravascular lithotripsy (IVL) has emerged as a novel therapy for the treatment of coronary calcification. Numerous studies have reported the safety and efficacy of IVL in calcified coronary lesions, while IVL balloon-uncrossable calcified coronary lesions are sometimes encountered in clinical practice. This case report represents a novel technique called "modified leopard-crawl" to advance the IVL balloon antegradely by creating significant calcium cracking forward when the enhanced stent visualization system reveals that the distal emitter seems adjacent to calcification. This technique might be a valuable alternative when conventional procedures fail and has the potential to enhance the clinical applicability of IVL in daily practice.

Diagnosis, Classification, and Management Strategies for Mitral Annular Calcification: A Heart Valve Collaboratory Position Statement.

Mitral annular calcium (MAC) with severe mitral valvular dysfunction presents a complex problem, as valve replacement, either surgical or transcatheter, is challenging because of anatomy, technical considerations, concomitant comorbidities, and advanced age. The authors review the clinical and anatomical features of MAC that are favorable (green light), challenging (yellow light), or prohibitive (red light) for surgical or transcatheter mitral valve interventions. Under the auspices of the Heart Valve Collaboratory, an expert working group of cardiac surgeons, interventional cardiologists, and interventional imaging cardiologists was formed to develop recommendations regarding treatment options for patients with MAC as well as a proposed grading and staging system using both anatomical and clinical features.

Removal of calciprotein particles from the blood using an adsorption column improves prognosis of hemodialysis miniature pigs.

Hyperphosphatemia is a major risk for poor prognosis in patients with end-stage renal disease. However, the molecular mechanism behind this link remains elusive. We and others have demonstrated that serum phosphorus levels correlate positively with circulating levels of calciprotein particles (CPPs). CPPs are colloidal mineral-protein complexes containing insoluble calcium-phosphate precipitates and have been reported to induce calcification in cultured vascular smooth muscle cells and inflammatory responses in cultured macrophages. Hence, we hypothesize that CPPs may be responsible for disorders associated with hyperphosphatemia. Using hyperphosphatemic miniature pigs receiving hemodialysis, here we show that removal of CPPs from the blood with a newly developed CPP adsorption column improves survival and alleviates complications including coronary artery calcification, vascular endothelial dysfunction, metastatic pulmonary calcification, left ventricular hypertrophy, and chronic inflammation. The present study identifies CPPs as an effective therapeutic target and justifies clinical trials to determine whether the CPP adsorption column may be useful as a medical device for improving clinical outcomes of hemodialysis patients.

Echocardiography Assessment of Rheumatic Heart Disease: Implications for Percutaneous Balloon Mitral Valvuloplasty.

Echocardiography is an important diagnostic imaging modality in recognizing rheumatic heart disease, a chronic sequelae of acute rheumatic fever. Left-sided heart valves, especially the mitral valve is typically affected, with stenosis or regurgitation as a consequence. Although assessment of valve area by 2D planimetry is the reference method for mitral stenosis severity, 3D planimetry provides more accurate measurement and diagnostic value. Careful selection of patients in terms of echocardiographic criteria is essential to ensure safety and success of the intervention and better long-term outcomes. Several echocardiographic scores based upon mitral valve mobility, thickening, calcification, and subvalvular thickening are developed to assess mitral valve anatomy and the feasibility of percutaneous mitral commissurotomy. 3D transesophageal echocardiography (TEE) provides detailed information of the mitral anatomy (commissural fusions, and subvalvular apparatus) before intervention. In addition, 3D TEE planimetry provides a more accurate measurement of the valve area compared with 2D echocardiography. Generally, huge annular calcification and lack of commissural fusion are unfavorable echocardiographic markers that increase the risk of complications and preclude the feasibility of percutaneous balloon mitral valvuloplasty. More contemporary prospective echocardiography research studies on patients with RHD from low- and middle-income countries are needed.

Intramuscular migration of calcium deposits into the deltoid muscle: two cases of a rare complication of rotator cuff calcific tendinopathy.

Rotator cuff calcific tendinopathy is a common non-traumatic shoulder pain condition that occurs predominantly in the supraspinatus tendon. Ultrasound-guided percutaneous irrigation of calcific tendinopathy (US-PICT) is a valid treatment in the resorptive phase. A complication of calcific tendinopathy is migration of calcium deposits outside the tendon. The most common site of migration is the subacromialsubdeltoid bursa (SASD). Another, but not frequent, type of migration is the intramuscular migration which mostly affects the supraspinatus, the infraspinatus and the biceps brachii muscles. This paper reports two cases of migration of calcification from the supraspinatus tendon to the deltoid muscle. The aforementioned site of migration has so far never been described in literature. Both patients presented calcification in the resorptive phase and therefore were treated by US-PICT.

Efficacy of therapeutic ultrasound in the treatment of chronic calcific shoulder tendinitis: a randomized trial.

BACKGROUND: Calcific shoulder tendinitis (CST) is characterized by hydroxyapatite crystals deposition in the rotator cuff tendons. Therapeutic exercises have been the mainstay of CST treatment, and evidence for therapeutic ultrasound (T-US) utilization and efficacy is lacking. AIM: This study aimed to determine whether 4500 J T-US combined with therapeutic exercises is superior to therapeutic exercises alone regarding calcification size reduction and symptom improvement in chronic symptomatic CST. DESIGN: This is a double-blind, placebo-controlled study. SETTING: This study was conducted at a University Department for Rheumatic Diseases and Rehabilitation of a University Hospital. POPULATION: Patients with chronic CST were analyzed. METHODS: After eligibility allocation, 46 patients with sonographically confirmed CST were divided into two groups (56 shoulders, 26 per group). Both groups performed the same therapeutic exercises for half an hour under physiotherapist supervision. In the treatment group T-US (4500 J, 10 minutes per session at a frequency of 1 MHz and an intensity of 1.5 W/cm2), and in the placebo group, sham T-US was applied for 4 weeks. Patients were assessed for: calcification size, shoulder pain, global health (GH), shoulder mobility (ROM), handgrip strength, Health Assessment Questionnaire Disability Index (HAQ-DI), Shoulder Pain and Disability Index (SPADI), and overall rehabilitation satisfaction. RESULTS: All assessed variables improved in both groups. A significantly greater reduction in calcification size was recorded in the treatment group compared to placebo: -10.92% (IQR 4.61% to 19.38%) versus -5.04% (2.30% to 7.22%), P=0.008. There was a significantly greater decrease in HAQ-DI, reduction of VAS GH, and an increase in hand grip strength in the treatment group, while no significant differences were observed for other parameters between the groups. CONCLUSIONS: Our results showed that adding the 4500 J T-US to therapeutic exercises in chronic symptomatic CST therapy resulted in greater calcification size reduction immediately following the treatment, as well as hand grip strength, HAQ-DI, and VAS GH improvement. CLINICAL REHABILITATION IMPACT: 4500 J T-US combined with therapeutic exercises is more effective in reducing calcification size than therapeutic exercises alone in the treatment of chronic symptomatic CST.

Intravascular lithotripsy for severe coronary calcification: a systematic review.

INTRODUCTION: Coronary artery calcification remains a challenge in percutaneous coronary interventions, due to the higher risk of suboptimal result with subsequent poor clinical outcomes. Intravascular lithotripsy is a novel way of treating severe coronary calcification as it has the ability to modify calcium both circumferentially as well as transmurally, facilitating stent expansion and apposition. We conducted a systematic overview of the published literature on intravascular lithotripsy (IVL) assessing the efficacy and feasibility of IVL in treating severe coronary calcification. EVIDENCE ACQUISITION: Of the retrieved publications, 62 met our inclusion criteria and were included. A total of 1389 patients (1414 lesions) with significant coronary calcification or under-expanded stents underwent IVL. EVIDENCE SYNTHESIS: The mean age was 72.03 years (74.7% male). There was a significant improvement in acute and sustained vessel patency, with mean minimal lumen diameter of 2.78±0.46 mm, resulting in acute gain of 1.72±0.51 mm. The acute procedural success rate was 78.2 to 100% with in-hospital complication rate of 5.6 to 7.0%. The majority of the studies reported 30-day MACE, which was between 2.2 to 7.8%. CONCLUSIONS: The recent studies have highlighted that the use of IVL with adjuvant intracoronary imaging has revolutionized the way of treating heavily calcified, non-dilatable coronary lesions and is likely to succeed the conventional ways of treating these complex lesions. We need further studies to gauge the long-term efficacy and safety of IVL against techniques currently available for calcium modification including conventional balloons, cutting or scoring balloons, rotational atherectomy and laser atherectomy.

Diagnosis and management of systemic sclerosis-related calcinosis.

INTRODUCTION: Calcinosis is common in patients with systemic sclerosis (SSc) and refers to the sub-epidermal deposition of calcium salts in the skin. SSc-related calcinosis is associated with significant morbidity, including through cutaneous ulceration and predisposition to become infected. AREAS COVERED: After briefly addressing aetiopathogenesis, we describe the clinical burden of SSc-associated calcinosis and provide a structured and practical clinical approach to diagnosis and assessment, including discussion of the role of different imaging modalities. The multi-faceted treatment of SSc-associated calcinosis is presented under three broad headings of 'general measures,' and 'medical treatment' and 'surgical treatment.' We adopted a narrative approach to identify relevant manuscripts to inform our review. EXPERT OPINION: SSc-related calcinosis is an area of major unmet clinical need and for too long has been a neglected area of research. Safe and effective treatments are badly needed to improve patient quality of life and outcomes. To facilitate future clinical trials, we require increased understanding of pathogenesis (to inform selection of potential targeted therapies) and reliable outcome measures, including those which will measure the impact and severity of calcinosis from the patient perspective. International collaborative research is ongoing to develop outcome measures and treatments for this potentially devastating complication of SSc.

Is conservative treatment a good choice for pediatric intervertebral disc calcification in children?

PURPOSE: Paediatric intervertebral disc calcification (PIDC) is a rare disease, and its aetiology remains unknown. This study aimed to analyse the characteristics and clinical outcomes of patients with PIDC. METHODS: After applying the exclusion and inclusion criteria, 159 children diagnosed with PIDC were analysed at our hospital between January 2010 and November 2020. Patients' demographic and clinical data were collected, such as sex, pain, duration time, physical examination, white blood cell count, erythrocyte sedimentation rate, C-reactive protein, and radiography, computed tomography, and magnetic resonance imaging findings. Patients were followed up for at least 6 months, and radiography or symptoms were evaluated. Fisher's exact test or χ2-test was used for statistical analyses. RESULTS: One hundred and fifty-nine patients were ultimately followed up with for about 12.5 ± 5.8 months. There were 103 male and 56 female, with an average age of 6.08 ± 2.62 years (2 months to 12 years). A total of 109 patients had only one PIDC, 29 patients had two PIDCs, and 21 patients had multiple PIDCs. Thirty patients were found incidentally and were asymptomatic. A total of 106 patients had neck torticollis. Sixteen patients had IDC herniations, fifteen patients had posterior longitudinal ligament calcification, two patients had anterior longitudinal ligament calcification, and 17 patients had herniation of the vertebral canal. All patients underwent conservative treatment, and none underwent surgery. All patients' symptoms resolved after either collar fixation or neck traction. CONCLUSION: PIDC can be treated conservatively, even when accompanied by herniation, longitudinal ligament calcification, or clinical neck symptoms. LEVEL OF EVIDENCE: IV.

Calcinosis in systemic sclerosis.

PURPOSE OF REVIEW: The aim of this study was to provide updated information on the prevalence, pathogenesis, diagnostics and therapeutics of calcinosis cutis associated with systemic sclerosis (SSc). RECENT FINDINGS: Observational studies show ethnic and geographical differences in the prevalence of calcinosis. In addition to clinical and serological associations, biochemical studies and in-vivo models have attempted to explain theories behind its pathogenesis, including prolonged state of inflammation, mechanical stress, hypoxia and dysregulation in bone and phosphate metabolism. Long-term use of proton pump inhibitors may increase the risk for calcinosis in SSc. Few single-centre observational studies have shown mild benefit with minocycline and topical sodium thiosulfate. SUMMARY: Calcinosis cutis is the deposition of insoluble calcium in the skin and subcutaneous tissues. It affects up to 40% of SSc patients and causes significant morbidity. Long disease duration, features of vascular dysfunction and osteoporosis have been associated with calcinosis. Altered levels of inorganic pyrophosphate and fibroblast growth factor-23 have been implicated in dysregulated phosphate metabolism that may lead to calcinosis in SSc. Plain radiography can help with diagnosis and quantifying the calcinosis burden. Surgical treatment remains the most effective therapy when feasible. At present, no medical therapies have proven efficacy in large randomized controlled trials.