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Skeletal Involvement

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
Overview
Bone Density
Bone Resorption
Costochondritis
Erosive Arthropathy

Foot Problems
Hand and Joint Involvement
Myositis (Muscle Inflammation)
Neurological
Osteoporosis
Tendon Friction Rubs (TFR)
Transverse Myelitis
Patient Stories

Overview of Musculoskeletal Involvement in Scleroderma

Treatment of Systemic Sclerosis Complications: What to Use When First-Line Treatment Fails—A Consensus of Systemic Sclerosis Experts. Symptoms treatment recommendations by over 100 scleroderma experts, for scleroderma renal crisis (SRC), digital ulcers (DU), pulmonary hypertension (PAH/PH), reflux, skin involvement, and arthritis. Seminars in Arthritis and Rheumatism Volume 42, Issue 1 , Pages 42-55, August 2012. (Also see: Scleroderma Renal Crisis, Digital Ulcers, Pulmonary Hypertension, Reflux, Skin Involvement, Scleroderma Treatments, and Dr. Janet Pope)
Correlates and responsiveness to change of measures of skin and musculoskeletal disease in early diffuse systemic sclerosis. Physician and patient global assessments have larger correlations with skin measures compared to musculoskeletal measures and skin variables were more responsive to change. PubMed, Arthritis Care Res (Hoboken), 2014 Apr 1. (Also see: Skin Involvement)
Lumbar canal spinal stenosis due to axial skeletal calcinosis and heterotopic ossification in limited cutaneous systemic sclerosis: successful spinal decompression Peripheral cutaneous calcinosis is well recognized in lcSSc, but axial skeletal calcinosis is less common. We present a case of lcSSc with lumbar canal spinal stenosis due to calcinosis and heterotopic ossification that had successful decompressive spinal surgery. Anita Weerakoon, Rheumatology, 28 August 2011 (Also see: Skeletal Involvement)

Bone Density

Bone Density or Bone Mineral Density (BMD) is a measurement of the compactness of the bone or the amount of bone mass per square centimeter. Bone density measurements are used to diagnosis osteopenia, osteoporosis and fracture risk. ISN.
Overview
Bone Density
Osteoporosis and Scleroderma
Treatments
Media Stories
Patient Stories

Bone Resorption in Systemic Sclerosis (Scleroderma)

Bone Resorption in systemic sclerosis (scleroderma) most commonly occurs in the fingertips and mandible (lower jaw). ISN
Overview of Bone Resorption in Scleroderma
Acroosteolysis
Dental Involvement
Causes of Bone Resorption
Treatments

Costochondritis

Costochondritis. Costochondritis is an inflammation of the cartilage that connects a rib to the breastbone (sternum). It causes sharp pain in the costosternal joint — where your ribs and breastbone are joined by rubbery cartilage. MayoClinic.

Erosive Arthropathy

Arthropathy is a disease of the joint.
Magnetic resonance imaging (MRI) versus musculoskeletal ultrasonography (MSUS) in detecting inflammatory arthropathy in systemic sclerosis (SSc) patients with hand arthralgia. SSc patients with arthralgia that have no obvious clinical inflammatory arthritis were found to have persistent inflammatory erosive arthropathy in their hands and wrists using MSUS and MRI. MRI is more sensitive than MSUS in this setting. PubMed, Rheumatol Int. 2013 Aug;33(8):1961-6.

Foot Problems in Scleroderma

Foot health needs in people with systemic sclerosis: an audit of foot health care provision. Foot health care and information are inadequate for people with SSc and foot problems, and preventative information is almost non-existent. Improved foot health information will better empower patients to self-manage low risk problems, and help identify high risk problems which require specialist care. Alcacer-Pitarch B. (PubMed) Clin Rheumatol. 2011 Jun 10.

Hand and Joint Involvement in Scleroderma

Hand and Joint involvement in scleroderma often affects the joints of the fingers, wrists, knees, and other joints. Hand involvement includes sclerodactyly, joint contractures, calcinosis, digital ulcers, Raynaud's, inflammation and carpal tunnel syndrome. ISN.
Overview
Sclerodactyly and Scleroderma
Joint Contractures
Joint Inflammation and Carpal Tunnel Syndrome
Treatments

Myositis (Muscle Inflammation)

The skin tightening may irritate the underlying muscles and tendons below the skin, causing myositis (inflammatory muscle disease), which often goes along with scleroderma. When this happens, a blood test will show elevation of muscle enzymes. Repeated blood tests for detection of muscle enzymes should be performed at your doctors discretion.

Once myositis is diagnosed, its very important to quit exercising, since repeated use of muscles will worsen the inflammation.

Outcomes of patients with systemic sclerosis-associated polyarthritis and myopathy treated with tocilizumab or abatacept: a EUSTAR observational study. In this observational study, tocilizumab and abatacept appeared to be safe and effective on joints, in patients with refractory systemic sclerosis. PubMed. Ann Rheum Dis. 2012 Dec 19.
Impaired skeletal muscle microcirculation in systemic sclerosis. BOLD MRI demonstrates a significant impairment of skeletal muscle microcirculation in systemic sclerosis (SSc). Arthritis Research & Therapy 2012, 14:R209.
Dermatomyositis and Polymyositis. Polymyositis is an inflammatory condition of the muscles ; dermatomyositis is an inflammatory condition of the skin and muscles, which causes a distinctive rash. ISN.

Neurological Involvement

Neurologic involvement in scleroderma: A systematic review. Previously considered a rare event, nervous system involvement in scleroderma has been increasingly recognized. Seizures and headache are the most reported features in LS en coup de sabre, while peripheral and autonomic nervous systems involvement predominate in systemic sclerosis (SSc). PubMed, Semin Arthritis Rheum. 2013 Jul 1.
Prospective study to evaluate the clinical and radiological outcome of patients with scleroderma of the face. Patients with localized scleroderma of the face have a high prevalence of neurological and ophthalmological changes. All cases of localized scleroderma of the face should be thoroughly examined for the presence of systemic changes. Science Direct, 06/19/2013. (Also see: Localized Scleroderma, and Eye Involvement)
Peripheral neuropathy: a complication of systemic sclerosis (Ssc). SSc patients have a considerable prevalence of pedal peripheral neuropathy as detected by loss of vibratory sensation and further studies are indicated to determine if routine screening for neuropathy and subsequent podiatric care for SSc patients with abnormalities can reduce pedal complications. PubMed, Clin Rheumatol. 2013 Feb 13.
Evaluation of abnormalities of orthostatic postural control in systemic sclerosis (SSc). This study seems to indicate a subtle neurophysiological dysfunction in the orthostatic postural control of female SSc patients. PubMed. Clin Exp Rheumatol.

Connection between scleroderma and the autonomic nervous system. Prolonged sensory chronaxy (the length of time needed to stimulate a muscle or nerve cell with an electric current) in the entire seemingly normal skin is a characteristic and pathognomonic (a particular sign whose presence means, beyond any doubt, that a particular disease is present.) feature of scleroderma. As a whole, the investigations suggest that in scleroderma the primary lesions concern the autonomic nervous system, in both the skin and the autonomic ganglia and centres. Journal of Neural Transmission.

Blink reflex discloses CNS dysfunction in neurologically asymptomatic patients with systemic sclerosis. The lack of overt trigeminal symptoms in our patients favours a suprasegmental dysfunction, possibly due to microvascular lesions disseminated in the subcortical white matter. PubMed. Clin Neurophysiol.
Rosie: Limited Systemic Sclerosis (Australia) Some of my symptoms may not be due to limited scleroderma, however most of these symptoms have appeared since my diagnosis...
Susie: CREST Syndrome I have had CREST syndrome for the past eight years. My specialist is Dr. Frederick Wigley, from Johns Hopkins...

Osteoporosis

Osteoporosis is when the bone density is low causing the bones to become porous and prone to fractures. In scleroderma, bone density can decrease due to certain medications, a decrease in exercise, change in diet and factors of the disease itself. ISN
Overview
Bone Density
Osteoporosis and Scleroderma
Treatments
Media Stories
Patient Stories

Tendon Friction Rubs

Tendon friction rubs are a red flag for serious internal organ involvement in diffuse scleroderma patients.
Joint and tendon involvement predict disease progression in systemic sclerosis (SSc): a EUSTAR prospective study. Joint synovitis and tendon friction rubs are independent predictive factors for disease progression in patients with early SSc and these easily detected clinical markers may be useful for the risk stratification of patients with SSc. PubMed, Ann Rheum Dis, 2014 Aug 27. (Also see: Hand and Joint Involvement)
The significance of palpable tendon friction rubs (TFR's) in early diffuse cutaneous systemic sclerosis (Ssc). Patients with early diffuse SSc having one or more TFRs are at increased risk of developing renal, cardiac and gastrointestinal involvement before and after their first Scleroderma Center visit, and have reduced survival so should be carefully monitored for serious internal organ involvement. PubMed, Arthritis Care Res. 2013 Jan 31. (Also see: Diffuse Scleroderma, Systemic Sclerosis: Prognosis and Mortality)

Transverse Myelitis

Transverse myelitis is a condition resulting from inflammation of your spinal cord. MayoClinic.
Transverse myelitis in systemic sclerosis (SSc). Transverse myelitis can occur as a rare manifestation of SSc and may respond favorably to immunosuppressive therapy. PubMed. Arch Neurol. 2004 Jan; 61(1): 126-8.

Patient Stories: Skeletal Involvement

Carpal Tunnel Syndrome
Alexandra: Scleroderma My scleroderma symptoms arrived in great style: reflux, itchy swollen skin, joint and muscle pain and tiredness. Plus hundreds of little red spots (telangiectasia). My mouth was tight and getting smaller too...
Annette: Diffuse Scleroderma (Illinois) I can see where having friends who understand what I am going through would be a big help...
Bill: Diffuse Scleroderma I was relieved to have a label for my condition but the doctor was not very reassuring telling me that there was no effective cure or treatment...
Claudia: Systemic Scleroderma Nature is wise, no doubt about it, when something is wrong with our body we have to pay attention...
Dawn M: Linear/Systemic Scleroderma My family and I were informed by the doctors, that the localized/linear form of scleroderma that I was diagnosed with, would never progress into the potentially fatal, systemic form...
Dee B: Limited Scleroderma/CREST Syndrome I also had the problem with people saying I was a hypochondriac, as at that stage all the doctors I saw found nothing wrong with me, but I constantly felt weary and ill...
Denise E: Diffuse Scleroderma I was first diagnosed with diffuse scleroderma in February 2002, but my symptoms began subtly in the summer of 2000...
Heather A: Scleroderma (South Africa) My fingers started to curl up and I could not wear any rings on my fingers anymore as they were so swollen...
Kathi: Scleroderma/Lupus I am forty-three years old and have not been feeling well for ten years...
Kerrie: CREST Syndrome with Limited Scleroderma If I was not stressed before I went in, I certainly was then. I am going to get referred on to see someone different. Hopefully, I will be luckier second time around...
Rhonda L: Systemic Scleroderma I have recently been diagnosed with sclerodema, and am still trying to wrap my brain around this...
Ro M: Scleroderma After six months of suffering, I have just been diagnosed with scleroderma. I was constantly tired, run down and plagued with infections...
Rosemary F: Surviving Daughter of Diffuse Scleroderma Patient She tried to explain it, but it was hard for me to comprehend the disease's symptoms. Mom said that it was the same thing that her oldest sister died from...
Sandy A: CREST Syndrome I have had surgery to remove the calcinosis on my knee and forearm twice, and once on my elbow. Yet it always comes back...
Sheri: CREST Syndrome In the winter of 1997, my fingers and toes started turning white and numb in response to the cold weather...
Sherrell: Possible Scleroderma When he examined me, he mentioned the possibility that I may have scleroderma. He said my fingers and face showed signs of this disease...
Shirley Wright: Limited Scleroderma I have had this disease for over twenty years now and I plan on being around a lot longer...
Star S: Morphea and Systemic Scleroderma I was diagnosed with morphea at age seven. Now at age thirty-three, I have recently developed systemic scleroderma with gastrointestinal manifestations, Sjogren's syndrome, inflammatory arthritis, malabsorption...
Soose: Limited Scleroderma I may have had scleroderma for the last year and a half or two years and did not know it...
Wes C.: Brother of Systemic Sclerosis Patient Just days ago, my sister Michelle was diagnosed with systemic sclerosis, after being misdiagnosed for almost two years...
Costochondritis
Brenda M: Primary Biliary Cirrhosis (PBC), Fibromyalgia, Pulmonary Fibrosis, and Sjogren's Syndrome In 1965, when I was twenty-six, I went to work for a firm of electro-platers, which was mainly aircraft work...
Dermatomyositis and Polymyositis
Personal Stories of Dermatomyositis and Polymyositis ISN.
Joint Contractures
Daniel B. Koch: Mixed Connective Tissue Disease with Scleroderma Two months after I was born the doctors noticed that my growth rate was very slow...
Karen M.R.: Diffuse Scleroderma and Fibromyalgia I was so glad she was there, because I sort of went into shock when the doctor said she was going to start me on chemo treatments...
Lisa V: Diffuse Scleroderma and Raynaud's When I first got sick, I thought I was dying. My fingers started hurting really bad...
Also see: Sclerodactyly (fingers curling inward) ISN
Joint Pain
Chela: Diffuse Scleroderma My facial expression changed a lot, but this went unnoticed to the people that do not know me very well...
(Español/Spanish) Chela: Esclerodermia Difusa Fue terrible para mí; las facciones de mi cara cambiaron mucho, pero sin ser notorio para las personas que no me conocen...
Dodo: Limited Systemic Scleroderma (Italy) The tips of my fingers turned cold, and then became cyanotic, always hurt and in the end they became ulcerated...
(Italiano) Dodo: Sclerodermia Sistemica Limitata (Italia) Ho iniziato ad avere alcune punte delle dita delle mani molto fredde, che in pochi giorni diventarono prima cianotiche, sempre piú doloranti e infine presentarono delle ulcere...
Jules: CREST She has left me with no answers or expectations and I'm afraid my obsession will only aggravate my symptoms...
Julie T: Surviving Daughter of a Diffuse Scleroderma Patient Our family deals with most difficult situations with laughter and humor, even in the most trying times...
Karligash: Systemic Scleroderma (Republic of Kazakhstan) Young, beautiful, full of hope and expectations for my life, for happiness and love — that was me, nineteen years of age...
(Russian) Карлыгаш: системная склеродермия (Республика Казахстан) Молодая,красивая,полная ожидания от жизни счастья,любви такая я была в 19 лет...
Kristi U: Systemic Scleroderma We sold our motel business because it hurt me so bad to work each and every day...
Leslie R: Scleroderma, Vitiligo, Lupus, Anemia, Hypertension and Type 2 Diabetes He told me that I have scleroderma and explained what this disease is about. After suffering so long I finally got some answers...
Theon: Scleroderma and Pneumothorax This is very hard for me, because I was a very active woman, and then suddenly I am totally and completely disabled...
Yvette: Undifferentiated Connective Tissue Disease (UCTD) and Thyroid Cancer For now, I have accepted that the diagnosis of my core problem is not important. The way I choose to deal with it is...
Peripheral Neuropathy
Craig R: CREST Scleroderma I had my first scleroderma symptoms at age twenty-two, which included severe Raynaud's with some ulceration and tissue loss...
Rotator Cuff Injury
Dawn: Confirmed Early Scleroderma I am awaiting surgery for what was thought to be a right rotator cuff injury. I now understand this may, in fact, be a symptom of my scleroderma...
 
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