Scleroderma


Article Author:
Talel Badri


Article Editor:
Anis Hariz


Editors In Chief:
William Gossman


Managing Editors:
Orawan Chaigasame
Carrie Smith
Abdul Waheed
Frank Smeeks
Kristina Soman-Faulkner
Benjamin Eovaldi
Radia Jamil
Sobhan Daneshfar
Saad Nazir
William Gossman
Pritesh Sheth
Hassam Zulfiqar
Navid Mahabadi
Steve Bhimji
John Shell
Matthew Varacallo
Ahmad Malik
Mark Pellegrini
James Hughes
Beata Beatty
Hajira Basit
Phillip Hynes


Updated:
1/20/2019 7:55:56 AM

Introduction

Scleroderma is a connective tissue disorder characterized primarily by the thickening and hardening of the skin. The combining form “sclero” means "hard" in Greek, and the word “dermis” means skin. There are two primary types of scleroderma: localized and systemic (also called systemic sclerosis). In localized scleroderma, the disease affects mainly the skin and may have an impact on the muscles and bones. In systemic scleroderma, there is an involvement of the internal organs, such as the digestive tract, heart, lungs, and kidneys, among others. The severity and outcome of scleroderma are variable.[1][2]

Etiology

The causes of scleroderma are not fully known. There is some evidence that genetic and environmental factors play a role in the genesis of scleroderma. Silica and certain organic solvents are recognized as risk factors of occurrence of systemic scleroderma. The result is an activation of the immune system, causing blood vessel damage and injury to tissues that result in scar tissue formation and the accumulation of excess collagen.

Genetic factors play at least a limited role. According to three US cohorts, the prevalence of the disease was 13 times higher in first-degree relatives of scleroderma patients than in the general population. OX40L gene polymorphism correlates with systemic scleroderma. IRF5 gene was found to correlate with systemic scleroderma as well as with the occurrence of interstitial lung disease during scleroderma.[3][4][5][6]

Epidemiology

Scleroderma is a rare disease. Its prevalence varies with ethnicity, gender, and geographic area. In France, the estimated incidence of systemic sclerosis was 158.3 per million in 2002, mostly limited to the cutaneous forms. The prevalence of the disease in Detroit was 242 cases per million adults with an annual incidence of 19.3 new cases per year per million adults over the period 1989-91. As in numerous other autoimmune diseases, women are at higher risk than men (ratio 4.6:1). Systemic scleroderma can occur at any age; however, it is rare in children and the elderly. The disease is most prevalent in individuals aged 30-50 years.

Localized scleroderma affects mostly women with an incidence of 3 cases per 100,000 individuals/year. Plaque form (also called morphea) is more prevalent in adults while linear scleroderma affects mostly children.[1][7][8]

Pathophysiology

Three primary mechanisms contribute to the development of the scleroderma: vascular anomalies, excess fibrosis, and autoimmune phenomenon.

Abnormal interactions between endothelial cells, fibroblasts, and lymphocytes (B and T) lead to microcirculatory vascular involvement. The endothelial cells produce large amounts of endothelin 1, causing vasoconstriction and fibroblast activation. Also, fibroblasts and activated endothelial cells produce reactive oxygen species that speed up vascular remodeling, leading to the obliteration of small vessels. Activated fibroblasts differentiate easily into myofibroblasts, which have increased collagen synthesis ability.[6][9]

History and Physical

There are two primary forms of scleroderma: localized scleroderma and systemic scleroderma also called systemic sclerosis (SSc).  

Localized scleroderma

Localized scleroderma is limited to the skin and underlying tissue. It generally evolves in three consecutive phases: edematous, indurated and sclerotic, and then atrophic. Its outcome is unpredictable and spontaneous improvements are possible. Different clinical forms of localized scleroderma can coexist in the same patient. There are two main groups of localized scleroderma: morphea and linear scleroderma.[1][10]

  • Morphea is the most common clinical form. It presents as a single or multiple plaques. These plaques are initially erythematous and then become sclerous, white, indurated, surrounded by a characteristic erythematous halo called “purple ring” reflecting its inflammatory activity. It is generally painless, but pruritus may be present. Later, hypopigmentation appears, and the lesions become generally atrophic. The lesions are mainly on the trunk and proximal extremities; the face is rarely affected. 

Plaque morphea can further classify into other sub-types, according to the shape or depth of the lesions. “Guttate” morphea or “white spot disease” refers to “drop-like”  shaped areas of skin involvement, whereas “subcutaneous” or deep morphea indicates a substantial involvement of deeper tissues with relative sparing of the overlying skin. The subcutaneous type may rarely extend deep into muscle tissues, but this does not indicate internal organ involvement.

Generalized morphea is defined as multiple morphea plaques, confluent or not, affecting more than two anatomical sites.

Bullous morphea is a rare sub-type characterized by blisters or erosions occurring on plaques of morphea.

  • Linear scleroderma manifests as thickened and indurated skin bands, which are most often on the face or extremities. It is the most common form of scleroderma in children. 

On the scalp or face, linear scleroderma gives an aspect called "en coup de sabre" (a French expression meaning “cut from a sword”). The sclerotic band is generally located on the forehead but can extend to the scalp (causing scarring alopecia), and to the nose as well as the upper lip. The skin is hypo or hyperpigmented, atrophic and adheres to the underlying bone. This form of scleroderma can sometimes be associated with ipsilateral hemiatrophy of the face and is therefore hardly individualizable from Parry-Romberg syndrome. 

Linear scleroderma of the limbs is called "monomelic" and often begins in childhood. Sclero-atrophic bands appear gradually and then extend to the muscles and tendons; this may lead to an extreme aspect of pansclerotic morphea with joint and bone deformities sometimes associated with a stop or growth retardation of the limb.

Systemic scleroderma or systemic sclerosis

This form of scleroderma is the most severe since it usually affects several internal organs. However, most patients do not have all the symptoms described below.

Raynaud’s phenomenon

Raynaud’s phenomenon (RP) is secondary to distal arteries vasospasm after exposure to cold or temperature changes. It is the most common manifestation of systemic sclerosis, occurring in more than 95% of patients. The typical RP crisis includes three phases: syncopal pallor with anesthesia limited to a few fingers, cyanosis paresthesia, and late erythematous, painful phase. Trophic disorders, mainly digital ulcerations, often complicate RP. About 50% of systemic scleroderma patients will have during the course of the of their disease at least one episode of digital ulceration. Digital ischemia can eventually result in distal gangrene that may result in auto-amputation. In more than 95% of patients with SSc, nailfold capillaroscopy shows vascular anomalies, such as architectural disorganization, giant capillaries, hemorrhages, loss of capillaries, angiogenesis and avascular areas.[11][12]  

Skin manifestations:

Skin lesions are bilateral and symmetrical, with a distal beginning, involving the fingers and sometimes the toes. Two clinical features should be distinguished [12][13]

  • Diffuse SSc, during which cutaneous sclerosis rises above the elbows and knees, which represents 30 to 40% of all patients.
  • Limited SSc, during which the cutaneous involvement limits itself to the fingers, hands, forearms, and face. Swelling of the fingers and hands may precede several months the onset of sclerotic lesions. Moreover, the skin of the fingers appears tense, giving a "sausage-like" appearance. The skin of the fingers becomes dry, thick and rough to the touch: this is called sclerodactyly, making it difficult to close the hand into a fist.

The nails diminish in size, curl and sometimes disappear. The face takes an inexpressive appearance, with a waxy skin in which the folds fade. The nose and the lips thin. Due to skin xerosis, small wrinkles form on the skin around the lips, which become finer. The limitation of oral opening may constitute a limitation for the consumption of solid foods, dental care, and other actions. The ability to open the oral cavity is measured by the inter-incisor distance. Telangiectasias are common during SSc and are usually localized in the face and mucous membranes.

Other cutaneous signs such as calcinosis, pigmentation disorders, and telangiectasia may be present.

Modified Rodnan skin score measures by palpation the extent and importance of cutaneous sclerosis in 17 zones of the body (0: normal cutaneous thickness; 1: minimal thickening; 2: moderate thickening; 3: thickening major). In diffuse forms, this score has a prognostic value, and usually reaches its maximum in the first three years after Raynaud's phenomenon and then tends to improve spontaneously.[14]

Pulmonary impairment

The pulmonary lesions include interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH). These two complications are the leading causes of death during SSc.  

  • Pulmonary arterial hypertension

Pulmonary arterial hypertension (PAH) occurs in up to 13% of patients with systemic scleroderma. It occurs more commonly in patients with limited cutaneous systemic sclerosis. By definition, PAH is pulmonary arterial pressure average greater than or equal to 25 mmHg measured at rest by right catheterization. PAH is suspected when pulmonary arterial systolic pressure exceeds 40 mm Hg at rest in echocardiography. Patients can remain asymptomatic for an extended time, especially if they do not have high levels of physical activity. Syncope, hemoptysis, and dysphonia (Ortner's syndrome) are signs of seriousness. An examination may find a systolic murmur of tricuspid insufficiency or diastolic murmur of pulmonary insufficiency, a loud second heart sound and signs of right heart failure.[15]  

  • Interstitial lung disease (ILD)

Interstitial lung disease (ILD) is common in patients with SSc since up to 90% of patients exhibit evidence of interstitial changes on high resolution computed tomography (HRCT). It is more frequent with the diffuse forms of the disease. Pulmonary auscultation reveals crackles in the bases with or without a dry cough and dyspnea. ILD assessment is by pulmonary function tests (PFTs) and HRCT. The most classic finding is the ground-glass, and then appear linear or reticular images, septal or intralobular, then a honeycomb appearance with traction bronchiectasis. SSc-ILD appears in the early years of the disease, and its most marked progression occurs in the first years after diagnosis. ILD progression is usually slow; however, about 12% of scleroderma patients will progress to terminal respiratory failure. An ILD involving more than 20% of the lung parenchyma correlates with poorer survival.[16][17][18]

Gastrointestinal manifestations

Gastrointestinal involvement in SSc concerns the whole digestive tract. Gastroesophageal reflux disease (GERD) occurs in 75 to 90% of patients. If a patient complains from dysphagia, erosive esophagitis, peptic stenosis, and endo-brachy-esophagus should be ruled out. Motor impairment can also affect the stomach, causing gastroparesis and more rarely watermelon stomach, a condition related to gastric antral vascular ectasia. In the small bowel, the motor impairment leads to bacterial overgrowth, with concomitant nutritional deficiencies (folate and vitamin B12), malabsorption (steatorrhea), and pseudo-obstruction. Anorectal involvement may cause fecal incontinence and rectal prolapse.[19]

Cardiac manifestations

They are common in SSc. However, only 15% of patients are symptomatic and then have a poor prognosis (estimated 2-year mortality: 60%). Cardiac involvement includes myocardial disease, conduction system defects, arrhythmias, or pericardial disease.  

Usually, only the coronary microcirculation is affected. In the case of pulmonary disease, right heart failure is possible. Pericardial involvement is quite common during SSc, usually without clinical consequence. Conduction disorders are quite rare. Rhythm disorders are detectable by Holter-electrocardiography.[20][21]

Renal involvement

Renal involvement is common in patients with SSc. The most serious manifestation is scleroderma renal crisis (SRC) which is a rare but major complication. It characteristically demonstrates the occurrence of sudden-onset hypertension and oligo/anuric acute renal failure. Clinical signs are those of malignant hypertension. SRC is more common in patients with diffuse SSc and having anti-RNA-polymerase III antibodies. Before the use of angiotensin-converting enzyme (ACE) inhibitors, SRC was the most common fatal complication of systemic scleroderma.[22][23]

Musculoskeletal Manifestations

Scleroderma may affect joints (arthritis, arthralgia), tendons (rubs, tenosynovitis) and muscles (myalgia, weakness, more rarely myositis). Friction tendon rubs, seen on the hands, knees, and ankles, indicate a poor prognosis. Digital retractions in flexion due to skin sclerosis and calcinosis, lead to major functional disorders.[24]

Evaluation

In patients with systemic scleroderma, the clinical assessment has two objectives: to confirm the diagnosis and to look for complications.

Complementary exams to be performed if systemic scleroderma is suspected are:  

  • Nailfold capillaroscopy
  • Screening for antinuclear antibodies (mainly anti-centromere and anti-scl70/anti-topoisomerase antibodies)
  • Transthoracic echocardiography 
  • HRCT of the chest
  • Diffusing capacity of the lung for carbon monoxide (DLCO) and spirometry
  • Hand X-ray
  • Esophageal manometry

When the diagnosis of systemic scleroderma is established, in addition to previous exams, it is necessary to perform:

  • Electrocardiogram
  • Complete blood count
  • Renal and liver functions 
  • Urinalysis 
  • NT-pro-BNP dosage
  • Upper gastrointestinal endoscopy

Exams that should be done for follow-up each year are:

  • NT-pro-BNP dosage
  • Spirometry and DLCO
  • Transthoracic echocardiography

Treatment / Management

Systemic Scleroderma

Treatment aims to relieve symptoms and to slow disease progression. No treatment can cure the disease. Therapeutic strategies should have their basis on a person’s symptoms and the need to prevent complications. The use of different immunosuppressive drugs remains disappointing. Immunosuppressive therapy is reserved for diffuse SSc of recent diagnosis (less than 3 to 5 years) and patients with progressive PID. Its basis is on induction with cyclophosphamide then a relay by mycophenolate mofetil.

The treatment of Raynaud's phenomenon is with calcium channel blockers. Protection against cold and smoking cessation are essential preventive measures. Patients should avoid drugs that worsen RP, in particular, beta-blockers (even as eye drops). In severe forms with trophic disorders, intravenous iloprost is a possible option. Bosentan has demonstrated efficacy in secondary prevention of digital ulcers.

The treatment of esophageal involvement is with prokinetic agents and proton pump inhibitors. Treatment of diarrhea related to microbial overgrowth is with an antibiotic such as amoxicillin, norfloxacin or sulfonamides.

ACE inhibitors have a significant place in the treatment of renal crisis but they are not recommended as prevention.

As for other forms of PAH, scleroderma-associated PAH may be treated with drugs designed to relax the walls of the pulmonary arteries, or vasodilation. Oxygen therapy and anticoagulants may be useful. Diuretics can help reduce swelling.

Corticosteroids may be useful in certain inflammatory manifestations of the disease as inflammatory joint manifestations and inflammatory myopathies.[11][15][16][16]  High doses of corticosteroids may induce scleroderma renal crisis.[17][19][22]

Localized scleroderma

Topical corticosteroids are the main treatment for plaque morphea. Generalized morphea may be treated with combination therapy of systemic steroids and methotrexate or with phototherapy. Linear scleroderma of the face or limbs generally requires the combination of systemic corticosteroids and methotrexate to avoid functional and/or esthetic disabilities.[1]

Differential Diagnosis

Other conditions may cause changes of the skin clinically close to those observed during scleroderma. The differential diagnosis includes:

  • Scleredema (scleredema diabeticorum and scleredema of Buschke) is characterized by skin thickening, affecting the neck, upper back, shoulders, and trunk. Raynaud phenomenon and internal organ involvement are absent.[25]
  • Eosinophilic fasciitis, often occurring following an unusual effort and characterized by edematous diffuse infiltration of the forearms and legs. It leads to the adherence of the skin to underlying fascia, usually sparing the hands and feet. Raynaud phenomenon is absent.
  • Eosinophilia-myalgia syndrome, which is a form of eosinophilia that is caused by L-tryptophan.Graft-versus-host disease, an autoimmune pathology that occurs as a result of bone marrow transplants in which the immune cells from the transplanted bone marrow attack the host's body.
  • Nephrogenic systemic fibrosis, which is usually caused by kidney failure. Skin changes may be indistinguishable from scleroderma, but Raynaud phenomenon is typically absent. May have internal organ fibrosis.

Prognosis

Life expectancy in patients with systemic sclerosis depends on the extent and severity of internal organ involvement. During SSc, the rate of death is 3.5 times higher compared to healthy subjects of the same age. The overall survival of patients with SSc is in the range of 75 to 80% after five years, 55% after ten years, 35 to 40% after 15 years and 25 to 30% after 20 years.  Factors correlating with a higher risk of death are advanced age at diagnosis, male patients, diffuse skin involvement, and visceral involvement (pulmonary, cardiac, renal). The pulmonary involvement is the main prognostic factor: five-year survival is greater than 90% in the absence of ILD and about 70% if ILD is present. The one-year survival is 55% in PAH associated with SSc. Autoantibodies also have a prognostic value: survival after ten years is 93% in the presence of anti-centromere antibodies, 66% in patients with anti-Scl70 and only 30% in patients with anti-RNA-polymerase III. The use of angiotensin-converting enzyme (ACE) inhibitors has transformed the prognosis of SRC, which remains an important cause of death.[7][26][27][28][29]

In localized scleroderma, the hardening of the skin generally ceases in the two years after the onset of the disease, and the lesions do not extend to other parts from the body. However, the disease can sometimes last several years, and some plaques may become more marked even after the end of inflammation. 

Complications

Skin hardening and musculoskeletal involvement in systemic scleroderma may cause functional disabilities, especially in hands. Oral opening limitations may alter food intake and dental care.

Linear scleroderma of the limbs may cause functional disabilities, particularly if the disease affects the underlying bone. Linear scleroderma “en coup de sabre” may induce esthetic and/or functional concerns.

Deterrence and Patient Education

Patients should be educated to stop smoking and avoid exposure to cold to prevent Raynaud's phenomenon. Using eye drops containing beta-blockers should also be avoided.

Enhancing Healthcare Team Outcomes

A multidisciplinary approach should include several medical specialties (dermatology, internal medicine, rheumatology, cardiology, pulmonology, gastroenterology, nephrology, radiology, biology) for the diagnosis and the treatment.

Because of the musculoskeletal impairment frequently observed in scleroderma patients, kinesitherapy is often needed to prevent definitive damages.


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Scleroderma - Questions

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In which of the following disorders are "onion skin" lesions in blood vessels most likely?



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A patient presents with severe reflux. Work up reveals scleroderma. The patient continues to be symptomatic despite medical therapy. What is the best surgical procedure for patients with scleroderma?



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Which of the following is not a feature of scleroderma?



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In which of the following disorders is one likely to see "onion skin lesions" in pathological sections?



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Anti-Scl-70 (Antitopoisomerase 1 antibody) may be positive in which of the following disorders?



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Raynaud phenomenon is most commonly associated with which medical disorder?



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A patient is diagnosed with Raynaud phenomenon. The patient later develops stiff, swollen, painful hands. She has had no weight loss, swallowing problems, or rashes. She does report achiness of multiple joints and fatigue. Her hands itch. The patient has non-pitting edema of the skin and difficulty making a fist. There are no other skin findings. Complete blood count and chemistry panel are normal. Antinuclear antibody (ANA) is positive at 1:640 with a nucleolar pattern. What is the most likely diagnosis?



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A 45-year-old female presents with a long history of general malaise and problems with her skin. Her blood work reveals the presence of anti-nucleolar and anti-centromere autoantibodies. What is the most likely diagnosis?



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Which organ is least likely to be involved with advanced systemic sclerosis?



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Which of the following medical disorders has renal lesions that are identical to those seen in malignant hypertension?



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What type of antibody in plasma can be seen in the diffuse form of scleroderma?



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A female presents with difficulty swallowing. On examination, the skin on the fingers appears tight and there is fibrous thickening of her skin. What is the most likely diagnosis in this patient?



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Which of the following tests is essential in a 33-year-old with scleroderma?



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A patient is presented to you with severe reflux. Work up reveals scleroderma. Patient continues to be symptomatic despite medical therapy. What is the best surgical procedure for patients with scleroderma?



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Why are patients with scleroderma particularly concerning during anesthesia?



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Which of the following is the best test to order in the case of a female who presents complaining of arthralgias, dyspepsia, and digital pain with cold exposure?



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Scleroderma is known to cause what type of lung disease?



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Which of the following features is characteristic of systemic scleroderma?



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Which of the following is true regarding scleroderma of the GI tract?



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Which of the following may be found in a patient with scleroderma?



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Which is not true with regards to scleroderma?



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On physical exam of a patient with scleroderma, the heart has an accentuated pulmonic second heart sound when auscultated. What may the patient have developed?



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What is a major cause of mortality in patients with scleroderma?



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Scleroderma - References

References

Careta MF,Romiti R, Localized scleroderma: clinical spectrum and therapeutic update. Anais brasileiros de dermatologia. 2015 Jan-Feb     [PubMed]
Denton CP,Khanna D, Systemic sclerosis. Lancet (London, England). 2017 Oct 7     [PubMed]
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Cutolo M,Sulli A,Secchi ME,Paolino S,Pizzorni C, Nailfold capillaroscopy is useful for the diagnosis and follow-up of autoimmune rheumatic diseases. A future tool for the analysis of microvascular heart involvement? Rheumatology (Oxford, England). 2006 Oct     [PubMed]
LeRoy EC,Medsger TA Jr, Criteria for the classification of early systemic sclerosis. The Journal of rheumatology. 2001 Jul     [PubMed]
Khanna D,Furst DE,Clements PJ,Allanore Y,Baron M,Czirjak L,Distler O,Foeldvari I,Kuwana M,Matucci-Cerinic M,Mayes M,Medsger T Jr,Merkel PA,Pope JE,Seibold JR,Steen V,Stevens W,Denton CP, Standardization of the modified Rodnan skin score for use in clinical trials of systemic sclerosis. Journal of scleroderma and related disorders. 2017 Jan-Apr     [PubMed]
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Goh NS,Desai SR,Veeraraghavan S,Hansell DM,Copley SJ,Maher TM,Corte TJ,Sander CR,Ratoff J,Devaraj A,Bozovic G,Denton CP,Black CM,du Bois RM,Wells AU, Interstitial lung disease in systemic sclerosis: a simple staging system. American journal of respiratory and critical care medicine. 2008 Jun 1     [PubMed]
Kirby DF,Chatterjee S, Evaluation and management of gastrointestinal manifestations in scleroderma. Current opinion in rheumatology. 2014 Nov     [PubMed]
Steen VD,Medsger TA Jr, Severe organ involvement in systemic sclerosis with diffuse scleroderma. Arthritis and rheumatism. 2000 Nov     [PubMed]
Clements PJ,Lachenbruch PA,Furst DE,Paulus HE,Sterz MG, Cardiac score. A semiquantitative measure of cardiac involvement that improves prediction of prognosis in systemic sclerosis. Arthritis and rheumatism. 1991 Nov     [PubMed]
Steen VD, Kidney involvement in systemic sclerosis. Presse medicale (Paris, France : 1983). 2014 Oct     [PubMed]
Mouthon L, Systemic sclerosis: views and thoughts for the future. Presse medicale (Paris, France : 1983). 2014 Oct     [PubMed]
Lóránd V,Czirják L,Minier T, Musculoskeletal involvement in systemic sclerosis. Presse medicale (Paris, France : 1983). 2014 Oct     [PubMed]
Badri T,Debbiche A,Koubaa W,Mokhtar I,Fenniche S, [A cutaneous infiltration: scleredema diabeticorum]. Presse medicale (Paris, France : 1983). 2011 Oct     [PubMed]
Lofgren S,Hinchcliff M,Carns M,Wood T,Aren K,Arroyo E,Cheung P,Kuo A,Valenzuela A,Haemel A,Wolters PJ,Gordon J,Spiera R,Assassi S,Boin F,Chung L,Fiorentino D,Utz PJ,Whitfield ML,Khatri P, Integrated, multicohort analysis of systemic sclerosis identifies robust transcriptional signature of disease severity. JCI insight. 2016 Dec 22     [PubMed]
Koh ET,Lee P,Gladman DD,Abu-Shakra M, Pulmonary hypertension in systemic sclerosis: an analysis of 17 patients. British journal of rheumatology. 1996 Oct     [PubMed]
Kawut SM,Horn EM,Berekashvili KK,Garofano RP,Goldsmith RL,Widlitz AC,Rosenzweig EB,Kerstein D,Barst RJ, New predictors of outcome in idiopathic pulmonary arterial hypertension. The American journal of cardiology. 2005 Jan 15     [PubMed]
Hudson M,Baron M,Tatibouet S,Furst DE,Khanna D, Exposure to ACE inhibitors prior to the onset of scleroderma renal crisis-results from the International Scleroderma Renal Crisis Survey. Seminars in arthritis and rheumatism. 2014 Apr     [PubMed]

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