Help researchers understand the relationship between systemic sclerosis and genetics. Eligible participants will get a 23andMe Health + Ancestry kit at no cost Skeletal muscle involvement is a common feature in systemic sclerosis (SSc) because muscle weakness is found in up to 90% SSc patients when systematically assessed. Muscle clinical, biological, and electromyographic features are similar to those of polymyositis or dermatomyositis, except for a higher proportion of mild symptoms Systemic sclerosis-associated myositis features minimal inflammation and characteristic capillary pathology Acta Neuropathol. 2021 Apr 17. doi: 10.1007/s00401-021-02305-3. Online ahead of print
The fatigue that accompanies scleroderma is not only an unwelcome, relentless symptom that is difficult to control and manage, but also can quite often be overlooked, with the initial management of the disease focussing on the more important (quite rightly), life threatening skin and organ fibrosis progression Hello everyone! Has anyone had Rituxinab infusions for muscle weakness? I tried IVIG without success. Now doc wants to try Rituxinab. I have systemic sclerosis and now they think with Myositis overlap. I really need to do some research on this. I'm already immunocompromised and this infusion will really do my immune system in. But I may have. Muscle weakness is a lack of strength in the muscles. They may not contract or move as easily as before. Some chronic medical conditions can cause the muscles to wear out more quickly or cause a..
Two female patients with anti-polymyositis (PM)-scleroderma (Scl) antibody-associated systemic sclerosis developed progressive proximal myopathy. Both patients had profound muscle weakness that was refractory to treatment with glucocorticoids with or without other oral immunosuppressive agents Nerves, muscles, and joints Many individuals living with systemic scleroderma develop muscle, joint, or nerve disease. Muscle involvement can lead to muscle pain, weakness, and muscle wasting (sarcopenia). Affected joints can be painful, stiff, and swollen Systemic sclerosis (SSc) is an autoimmune inflammatory condition. It results in potentially widespread fibrosis and vascular abnormalities, which can affect the skin, lungs, gastrointestinal tract, heart and kidneys. The skin becomes thickened and hard (sclerotic)
Systemic Sclerosis (Scleroderma) Systemic Sclerosis (SSc) or Scleroderma is a rare disease characterized by hardening of the skin due to a build-up of fibrous scar tissue, causing a tight and shiny appearance. The most common area affected by the disease is the fingers, however many other areas can be affected Male in his early 80s with hx of pulmonary tuberculosis treated with RPIE, non ST elevation myocardial infarction, overlap syndrome with polymyositis and limited systemic sclerosis (+PM-SCL), ILD, patulous esophagus with GERD is presenting to LBJ ED with progressive dysphagia and generalized weakness. On review of his past history, five years. Other symptoms of systemic scleroderma include hair loss, calcium deposits under the skin known as calcinosis, small and dilated blood vessels under the skin's surface called telangiectasias, joint pain, muscle weakness, shortness of breath, dry cough, diarrhea [sclerodermanews.com] Nausea Systemic scleroderma, or systemic sclerosis, is an autoimmune rheumatic disease characterised by excessive production and accumulation of collagen, called fibrosis, in the skin and internal organs and by injuries to small arteries.There are two major subgroups of systemic sclerosis based on the extent of skin involvement: limited and diffuse. The limited form affects areas below, but not above.
. Resources Available BACKGROUND: Individuals with systemic sclerosis have muscle functions with varying degrees of muscle weakness and atrophy, which in turn can have a negative impact on functional and health-related quality of life. This study aimed to evaluate peripheral muscle performance using isokinetic dynamometry of the knee in women with systemic sclerosis. Individuals with systemic sclerosis have muscle functions with varying degrees of muscle weakness and atrophy, which in turn can have a negative impact on functional and health-related quality of life. This study aimed to evaluate peripheral muscle performance using isokinetic dynamometry of the knee in women with systemic sclerosis and to.
Aches and pains in several joints often accompany early symptoms. Sometimes inflammation of the muscles , with its accompanying muscle pain and weakness, develops. Skin changes. Systemic sclerosis can damage large areas of skin or only the fingers (sclerodactyly). Sometimes systemic sclerosis tends to stay restricted to the skin of the hands For example, dysphagia may accompany weakness in inclusion body myositis and systemic sclerosis, whereas menorrhagia may attend the weakness that occurs in hypothyroidism Systemic sclerosis is considered to be an autoimmune process, possibly resulting from deleterious effects of cytokines on extracellular matrix proteins and tissue healing. Some studies have also implicated cellular microchimerism in its pathogenesis. Muscle weakness, cutaneous pruritus, and Raynaud phenomenon are common. Renal failure can.
Although the prevalence of muscle weakness in the general population is uncertain, it occurs in about 5% of U.S. adults 60 years and older. Determining the cause of muscle weakness can be challenging So she changed my diagnosis to systemic sclerosis and I came back home thinking things would improve. He agreed although feels this is less likely as I don't report muscle pain, just resting weakness. So I'm now waiting for him to call on 22nd if this month for MG test results. I think personally that the eye issues are another unusual. There are two main types or classes of scleroderma: localized scleroderma, which affects only certain parts of the body and includes linear scleroderma and morphea, and systemic sclerosis, which affects the entire body. Scleroderma is a symptom of several rheumatic diseases (conditions characterized by inflammation and pain in the muscles. Myasthenia gravis is a chronic autoimmune, neuromuscular disease that causes weakness in the skeletal muscles that worsens after periods of activity and improves after periods of rest. These muscles are responsible for functions involving breathing and moving parts of the body, including the arms and legs. The name myasthenia gravis, which is. Weakness of the muscles of the face (Bell palsy) Group of disorders involving brain and nervous system functions (cerebral palsy) Nerve inflammation causing muscle weakness (Guillain-Barre syndrome) Multiple sclerosis. Pinched nerve (for example, caused by a slipped disk in the spine) Cancer. Cerebrovascular disease
Leg weakness and fatigue. Technically, fatigue and weakness have 2 different definitions. Weakness is a lack of muscle or physical strength and the feeling that extra effort is needed to move. Fatigue is a feeling of tiredness or exhaustion because of a lack of energy or sleep.But in these cases, I feel like they are one and the same Systemic sclerosis is characterised by progressive accumulation of collagen, It presents with muscle weakness, elevated muscle enzymes and a rash affecting the face, chest, arms and hands; Dermatomyositis of the eyelids is known as heliotrope rash Objective muscle weakness is present sometime during systemic sclerosis in at least 80% and is usually multifactorial (95; 27). Disuse and deconditioning are responsible in part. Over half demonstrate a mild, nonprogressive myopathy with slight elevations of serum creatine kinase and aldolase levels Systemic sclerosis can cause the tissues around joints to stiffen, which can reduce the range of movement of joints. It can also cause pain and swelling around affected joints. Muscle weakness is also sometimes a symptom of systemic sclerosis. People who have had systemic sclerosis for a long time may find tha Progressive Systemic Sclerosis (Scleroderma) By THOMAS A. MEDSGER, JR., GERALD P. RODNAN, JOHN Mooss~ AND JOHN W. VESTER 53 patients with typical PSS were exam- ined for evidence of muscle weakness, abnormal serum enzymes, disturbances in urinary creatine and creatinine, and histopathologic alterations in skeletal muscle tissue
Systemic sclerosis is characterized by generalized micro and macroangiopathy which may potentially impact RDW which was found to be associated with pulmonary hypertension, and with severe proximal muscle weakness on Medsger score compared to those without muscular weakness, which is in accordance with Peng et al.. Systemic sclerosis, also called scleroderma, is an immune-mediated rheumatic disease that is characterised by fibrosis Lower-limb swelling and muscle weakness or fatigue might be reported, especially in early-stage diffuse cutaneous systemic sclerosis.6 Weight loss, ofte Patients with diffuse systemic sclerosis (dSS) with an associated ANCA-positive vasculitis have been infrequently described. In this article, we report the case of a 53-year-old woman, previously diagnosed with dSS, who developed an ANCA-associated vasculitis, which manifest as peripheral nerve and muscle disease Systemic sclerosis (SSc) is an immune-mediated rheumatic disease characterized by fibrosis of the skin and internal organs and vasculopathy. 4 The prevalence of musculoskeletal involvement in SSc is not completely known and varies from 13% to 96% depending on the series published. 5, -, 10 Muscle weakness in patients with SSc, when present. Objective. Although patients who develop systemic sclerosis (SSc) later in life (≥ 65 yrs) may express the entire clinical spectrum of disease, we hypothesize that patients with late-age onset SSc incur a different risk for specific organ manifestations of disease compared to those with early-age onset SSc. Methods. In total, 2300 patients with SSc were evaluated between 1990 and 2009 and.
Inflammatory Ocular Myopathy in Systemic Sclerosis (Scleroderma) A Case Report and Review of the Literature Frank C. Arnett, MD,Ronald G. Michels, Baltimore A patient with systemic sclerosis (sclero- derma) had diplopia. On the basis of results from ophthalmologic examination, muscle enzyme studies, and muscle biopsy, he was found to have ocular myositis, as well as an asymptomatic proximal. Systemic sclerosis is an autoimmune connective tissue disease, which is characterised by immune dysregulation and progressive fibrosis that typically affects the skin, with variable internal organ involvement. It is a rare condition that affects mostly young and middle-aged women, resulting in disproportionate morbidity and mortality. Currently, interstitial lung disease is the most common.
Respiratory muscle weakness due to neuromuscular disease: Clinical manifestations and evaluation; Society guideline links: Systemic sclerosis (scleroderma) Tests of respiratory muscle strength; Treatment and prognosis of interstitial lung disease in systemic sclerosis (scleroderma) Treatment of idiopathic pulmonary fibrosi Systemic sclerosis is likewise called scleroderma, progressive systemic sclerosis, or CREST syndrome. CREST represents: calcinosis; Extra symptoms include chest pain or swelling of the legs. Some patients have cardiomyopathy or weakness of the heart muscle, and others might experience unusual cardiac rhythms.. . The study, Correlation Between Skin and Affected Organs in 52 Sclerodermic Patients Systemic sclerosis (SSc) is an immune-mediated rheumatic disease characterized by ﬁbrosis of the skin and internal organs patients recovered muscle weakness completely; in fact, scapular winging,whenpresent,didnotimprove,andthereforelimitation to extend the arms persisted over time
. AU - Paik, Julie J. AU - Wigley, Fredrick M. AU - Shah, Ami A. AU - Corse, Andrea M. AU - Casciola-Rosen, Livia. AU - Hummers, Laura K. AU - Mammen, Andrew L Systemic sclerosis (also sometimes referred to as scleroderma, systemic scleroderma, or CREST syndrome) is an autoimmune sclerosing disease of the skin and the internal organs. Autoimmune diseases occur when the immune system, which normally protects you from infections and cancer, makes a mistake and begins to attack parts of your body
Clinical Presentation of Diffuse Systemic Sclerosis. massive depositing of collagen in microvascular multi-system disease diagnosis typically involves ruling out other conditions. progressive and symmetric muscle weakness difficulty with everyday tasks rash. Signs and Symptoms of Polymyositis and Dermatomyositis. Raynaud's Phenomenon. Anti-Ku antibodies were identified in an adolescent patient with systemic sclerosis (SSc)-polymyositis overlap syndrome, according to a case report published in Pediatric Dermatology.A 13-year-old girl presented with symmetric proximal muscle weakness that had developed over several years and was associated with tightness and shininess of the skin on her arms, legs, and anterior upper chest. Figure 1. Clinical features at disease onset in 153 children with systemic sclerosis. Data from the Pediatric Rheumatology European Society international database . MCP: Metacarpophalangeal joint. 0 10 2030 405060 7080 Abnormal chest x-rays Calcinosis Muscle weakness Dyspnoea Digital infarcts Nailfold capillaries changes Arthritis Weight. AISH MEDICINE 3A [IMMUNO/RHEUMA] : Idiopathic Inflammatory Myopathies, Systemic Sclerosis and Mixed CT Disease PPT, Harrison 's Principle s of Internal Medicine 20 th FEU-NRMF Batch 2022 IDIOPATHIC INFLAMMATORY MYOPATHIES (IIM) Heterogenous group of disorders Characterized by: • Symmetrical proximal muscle weakness • Elevated serum levels of muscle enzymes: CPK, aldolase, ALT, AST, LDH. Systemic sclerosis (SSc) is a multisystem autoimmune disorder characterized by vascular injuries and fibrosis of the skin and various internal organs. Th2 polarized immune responses have been shown in the early and active stage of SSc (1). Due to the clinical features of muscle weakness and elevated muscle enzymes, we suspected that she.
Systemic sclerosis requires organ or tissue involvement in addition to skin changes. This involvement may be manifested as dysphagia, gastroesophageal reflux, dyspnea, palpitations, arthritis, muscle weakness, and neuropathies Valid for Submission. M34.82 is a billable diagnosis code used to specify a medical diagnosis of systemic sclerosis with myopathy. The code M34.82 is valid during the fiscal year 2021 from October 01, 2020 through September 30, 2021 for the submission of HIPAA-covered transactions involves muscle weakness and fatigue improves with rest. Common in young women and old men. Signs and symptoms of Myasthenia gravis? ptosis, diplopia, difficulty chewing, limb weakness, respiratory difficulties Systemic sclerosis (scleroderma) Part 1. Systemic sclerosis (scleroderma) Part 2. Systemic sclerosis (scleroderma) Part 3 Systemic sclerosis (SSc) is a rare autoimmune inflammatory disease characterized by vasculopathy and fibrosis in skin and internal organs. The disease is commonly divided into two subtypes with regard to the extent of skin involvement: limited cutaneous (lcSSc) or diffuse cutaneous systemic sclerosis (dcSSc) ().The clinical course can vary from limited skin thickening to severe organ damage. Bilateral diaphragmatic muscle weakness and paralysis. (88%) Evidence of active muscle disease that cannot be controlled with standard post-transplant immunosuppression, or progressive muscle weakness causing severe frailty and unable to perform pre-and post-transplant pulmonary rehabilitation. (98%) Musculoskeletal/ Respiratory muscle
‡Active disease was defined as a score >3 by calculating European Scleroderma Study Group disease activity indices for systemic sclerosis proposed by Valentini et al. 45 §Immunosuppressive therapy was defined as treatment with corticosteroids (prednisone dose ≥2.5 mg/day or other dosage forms in equal dose) or any immunosuppressant Abstract. Objectives: The aim of this study is to investigate the effects of respiratory muscle strength and pulmonary involvement on functional status, fatigue, and health related quality of life (HRQoL) in patients with systemic sclerosis. Patients and methods: Thirty-three patients (2 males, 31 females; mean age 46.2±13.4 years; range 27 to 77 years) with systemic sclerosis and 34 healthy. Systemic sclerosis (SSc) is an autoimmune systemic disease characterized by small vessel involvement muscle weakness. Carpal tunnel syndrome and tendon friction rubs are due to the fibrotic thickening of the tendon sheaths. Muscle weakness occurs just in 5% of the lcSSc patient
Systemic sclerosis (SSc) is an autoimmune disorder which can affect nearly every body organ. Muscle involvement is one of the most serious manifestations of SSc. It can present itself in a wide range of pathologies. It can be as indolent as Systemic Sclerosis is a chronic condition caused by excessive secretion of collagen inside the body. Some consider it a chronic condition and weakness of muscles with high level of muscle enzymes in blood. Muscle weakness due to the illness and lack of use due to joint injury, malnutrition, muscle fibrosis or the use of certain drugs. Systemic sclerosis (SSc) is an autoimmune disease initially recognized by hand involve-mentduetocharacteristicRaynaud'sphenomenon(RP),puffyhands,skinthickening,and contractures resembling claw deformities. SSc contributes to hand impairment through muscle weakness, new DU, and decreased left heart function [3, 4] Maurer, B Muscle involvement in systemic sclerosis and scleroderma-myositis overlap syndromes. In: Hachulla E, Czirják L, eds. EULAR Textbook on systemic sclerosis 1st ed. London: BMJ Publishing Group 2013;265-273. Google Schola Lack of exercise is a common cause of fatigue, weight gain, and muscle weakness. Hypocalcemia. Hypocalcemia is a blood condition marked by tingling, muscle spasms and aches, and confusion. Multiple sclerosis. Multiple sclerosis is a disease of the nervous system causing difficulties with balance, speech, and movement
This idiopathic systemic autoimmune disease has clinical and serological features shared between systemic sclerosis, systemic lupus erythematosus the upper back and the 'V-neck sign' when involving the chest wall. 9 The cutaneous manifestations of DM may precede muscle weakness and sometimes occur without it Systemic muscle weakness is a rare adverse event occurring with administration of botulinum toxin A. Based on data from the identified cases of systemic muscle weakness with botulinum toxin A, this adverse event was more common in female and younger patients and those with spasticity or neurogenic detrusor overactivity indication or those receiving abobotulinum toxin A formulation and/or. Muscle weakness is commonly due to lack of exercise, ageing, muscle injury or pregnancy. It can also occur with long-term conditions such as diabetes or heart disease . There are many other possible causes, which include stroke, multiple sclerosis , depression, fibromyalgia and chronic fatigue syndrome (ME)
Respiratory compromise can occur in systemic sclerosis solely on the basis of restriction in chest wall expansion in the absence of intrinsic lung disease and that this may have serious consequences. Respiratory muscle weakness with hypercapnic respiratory failure in systemic sclerosis has rarely been described in the literature, but the. Abstract: Skeletal muscle involvement is a common feature in systemic sclerosis (SSc) because muscle weakness is found in up to 90% SSc patients when systematically assessed. Muscle clinical, biological, and electromyographic features are similar to those of polymyositis or dermatomyositis, except for a higher proportion of mild symptoms
Systemic sclerosis (SSc) is a rare disease of connective tissue with the potential to affect various organs in the body. Skin, often being the initial organ to develop significant changes due to the disease, the patient may be presenting for the first time to dermatologists. Proximal muscle weakness: 3 (9.4) Elevated serum lactate. Histological assessment of myopathy in systemic sclerosis Muscle biopsy can be a helpful diagnostic and management tool in SSc-associated myopathy, particularly if the diagnosis is unclear. In a study of 42 SSc patients with a myopathy, muscle histopathology was studied to identify whether unique subsets existed in SSc muscle disease Systemic Sclerosis, also called Scleroderma, is a chronic multi-system disease that is characterized by an early inflammatory phase followed by progressive fibrosis and small-vessel vasculopathy. Two clinical subtypes, Limited and Diffuse have been described, differing the geography of affected tissues. Etiology and Pathogenesis Systemic BL Insidious Genetic testing Muscle biopsy: Progressive proximal lower limb weakness with calf pseudohypertrophy in early childhood. Gower sign positive. Multiple sclerosis exacerbation + + + + Generalized Systemic NL Sudden ↑ CSF IgG levels (monoclonal) Clinical assessment and MRI: Blurry vision, urinary incontinence, fatigu Diffuse cutaneous systemic sclerosis (dcSSc) is a subtype of systemic scleroderma (systemic sclerosis) characterized by skin hardening (fibrosis) and problems in many organs of the body. The disease can occur at any age but mainly affects people between 40 and 50 years of age. Symptoms include Raynaud's phenomenon; skin fibrosis beginning on the fingers and face that rapidly becomes.
Systemic sclerosis, also known as scleroderma, is a rare disease characterized by the thickening and scarring of connective tissue of multiple organs in the body. The scarring can also affect the. Introduction. Systemic sclerosis (SSc), a rheumatic disease characterised by autoimmunity, tissue fibrosis and vasculopathy, has a high mortality rate compared with other rheumatic diseases.1 2 Mortality in SSc is the result of organ involvement, with lung disease (either interstitial lung disease or pulmonary arterial hypertension (PAH)) being the most prominent risk factor for death.3 Skin. IM-VAMP is most common form of inflammatory myopathy. May present with proximal weakness or high serum CK. NT5C1A antibodies (64%) Mortality. Rate: 1.7. Most common cause of death: Aspiration due to dysphagia (20%) Genetics. Increased frequency of FYCO1 missense variants (11% vs 2.6% in controls) 100 systemic sclerosis; disease activity; outcomes research; We read with great interest the article 'Development and validation of the Scleroderma Clinical Trials Consortium Damage Index (SCTC-DI): a novel instrument to quantify organ damage in systemic sclerosis' by Ferdowsi et al.1 We commend the authors' to have come up with the much-needed concept of a damage index in systemic sclerosis
Likewise, muscle inflammation sometimes causes weakness instead of pain. Causes of myalgia and/or myositis range from localized muscle injury to systemic disorders. Localized muscle injury includes overexertion (such as after unaccustomed exercise or following the intense contractions of a seizure), trauma to the muscle by over stretching or. A number of conditions that affect the muscles or nerves in the body can produce leg weakness. These conditions include: Amyotrophic lateral sclerosis (ALS, also known as Lou Gehrig's disease; severe neuromuscular disease that causes muscle weakness and disability). Cerebral palsy (disordered brain function that leads to a variety of neuromuscular symptoms Systemic sclerosis happens when your tissue makes too much collagen and other proteins. Collagen is a protein that helps give structure to your skin, nails, hair, tendons, and other tissue. Sometimes, muscle pain and weakness. Systemic sclerosis can cause too much scarring to build up in other organs of the body, such as the joints, lungs.
original article Lower limb muscle strength is associated with functional performance and quality of life in patients with systemic sclerosis Tatiana R. L. Lima1, Fernando S. Guimarães1,2, Mara N. Carvalho3, Thaís L. M. Sousa1, Sara L. S. Menezes1,2, Agnaldo J. Lopes1,4 ABSTRACT | Background: Complaints of peripheral muscle weakness are quite common in patients with systemic sclerosis (SSc) Patients with systemic sclerosis (SSc) can have muscle involvement in the form of myositis or non-inflammatory myopathy. The muscle involvement can be associated with left ventricular dysfunction (LVD) in patients with SSc, resulting in worse prognosis. Muscle weakness, reported by the patient and evidenced as muscle strength deficiency on.
Upon careful examination, 23 of 24 patients with progressive systemic sclerosis (PSS) were found to have abnormalities of muscle. Nineteen patients presented a homogeneous pattern of muscle abnormalities, which untreated was associated with a stable course (simple myopathy). Three patients demonstrated inflammatory muscle disease indistinguishable from polymyositis while a fourth patient. Systemic sclerosis (SSc) is a systemic autoimmune disease that is characterised by endothelial dysfunction resulting in a small-vessel vasculopathy, fibroblast dysfunction with resultant excessive collagen production and fibrosis, and immunological abnormalities. The classification of SSc is subdivided based on the extent of skin involvement into diffuse cutaneous sclerosis (dcSSc), limited. Progressive Systemic Sclerosis -ANA -SCL-70 antibodies. Localized Scleroderma primarily in extremities no internal organ involvement usually does not require treatment Muscle weakness Objective symmetric weakness, usually progressive, of proximal upper extremities 0.7 0.7 Objective symmetric weakness. To evaluate the clinico-serological profile and to assess diagnostic parameters of myopathy in patients with systemic sclerosis (SSc)-associated myopathy. METHODS: We explored the profiles of SSc-myopathy patients and matched non-myopathy SSc patients as well as different diagnostic measures for muscle affection
Objective proximal muscle weakness was noted in only 10% of patients [24, 25]; patient-reported weakness, however, was more frequent as ~33% patients reported subjective muscle weakness . In the European Scleroderma Trials and Research (EUSTAR) group cohort and other cohorts, 27-37% of patients with diffuse cutaneous SSc reported muscle. Norwood, Massachusetts-based Corbus Pharmaceuticals' shares plunged 77% at news the company's lenabasum failed to hit the primary endpoint in the Phase III RESOLVE-1 trial of diffuse cutaneous systemic sclerosis (SSc).. SSc is a rare and life-threatening multi-system autoimmune disease. Diffuse cutaneous SSc is a subtype of systemic sclerosis marked by hardening and scarring (fibrosis) of. juvenile scleroderma, juvenile systemic sclerosis, systemic sclerosis, outcome, prognosis, organ involvement, diffuse subtype, limited subtype, antibody profile, EUSTAR Introduction Juvenile SSc (jSSc) is a rare disease in childhood, with an estimated incidence of 0.05 per 100 000 [ 1 ] and a prevalence of ∼1 in 1 000 000 Systemic sclerosis (SSc) is a systemic connective tissue disease of unknown etiology that affects the skin, blood vessels, muscles, joints, and a variety of internal organs, such as the digestive. Systemic Autoimmune Diseases. Whereas organ-specific autoimmune diseases target specific organs or tissues, systemic autoimmune diseases are more generalized, targeting multiple organs or tissues throughout the body. Examples of systemic autoimmune diseases include multiple sclerosis, myasthenia gravis, psoriasis, rheumatoid arthritis, and systemic lupus erythematosus Proximal muscle weakness, DLCO, and LV diastolic dysfunction may identify patients at risk for progressive LV systolic dysfunction and in need of closer cardiac monitoring. Cardiac involvement is a main cause of mortality in systemic sclerosis (SSc)