Scleroderma involves multiple systems. Limited cutaneous systemic sclerosis was earlier described as the CREST syndrome which included calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly and telangiectasis.

Scleroderma leads to tightening and hardening of the skin with beaklike facial feature and lack of wrinkles. It also leads to digital ulceration, loss of skin creases and contractures. Hypopigmented or hyperpigmented patches are seen on the skin.

It affects the vascular system and Raynaud’s phenomenon is seen as the first presenting symptom. Other symptoms include healed pitting ulcers in the fingers tips and telangiectasis. It also affects the gastrointestinal system and causes gastroesophageal reflux.

Respiratory affections are presented in the form of increasing dyspnea, pulmonary hypertension leading to chest pain and dry persistent cough suggestive of restricted lung disease. It also causes weakness and pains in the joints and muscles with contractures which restricts the range of movement of the joints [17].

The cardiovascular system presents with dyspnea caused by pericardial effusion or congestive heart failure or myocardial fibrosis. The other symptoms include sicca syndrome with poor dentition.

It also causes renal complaints like renal hypertension, renal crisis or renal insufficiency. Neurologic affections include facial pain and reduced sensations due to trigeminal neuralgia. One can suffer from carpel tunnel sensory neuropathy causing paresthesia and weakness of the hand.

Patients with diffuse systemic sclerosis may suffer from oropharyngeal and oesophageal cancers [18] [19] [20].

• Weakness

  Arthritis and muscle weakness, including: Pain, stiffness and swelling General fatigue Muscle weakness, often in your upper arms or thighs Digestive problems including poor function of your esophagus and bowels. [ucsfhealth.org]

  Other signs are symptoms of carpal tunnel syndrome or muscle weakness. [sclerodermasociety.co.uk]

  A 66-year-old woman with a history of interstitial lung disease presented with a 3-month history of dropped head syndrome (DHS), followed by camptocormia and extremity weakness. [ncbi.nlm.nih.gov]

  It also causes weakness and pains in the joints and muscles with contractures which restricts the range of movement of the joints. [symptoma.com]

  Case Report A 55-year-old female patient was admitted with weakness and fatigue. Elevated liver enzymes (ALT 168 IU, AST 126 IU) were detected. [causapedia.com]

• Fatigue

  Symptoms may include: Fatigue Severe headache Shortness of breath Trouble seeing Mental confusion Reviewed by health care specialists at UCSF Medical Center. [ucsfhealth.org]

  Early symptoms of pulmonary hypertension include shortness of breath, chest pain, and fatigue. How common is scleroderma? Approximately 250 persons per million American adults are affected by scleroderma. [my.clevelandclinic.org]

  Symptoms Symptoms of systemic scleroderma may include: Acid reflux Dry mouth Fatigue Joint stiffness and pain Raynaud’s phenomenon, in which the fingers or toes change color, often in response to cold Skin tightening and thickening Shortness of breath [iuhealth.org]

  His primary complaints are dysphagia with frequent regurgitation, chronic melena, myalgias, pruritus, progressive fatigue and dyspnea on exertion, and painful swelling of his fingers with occasional discoloration in his fingertips. 1. [stanfordmedicine25.stanford.edu]

  Fatigue and Depression Severe, if not debilitating, fatigue, accompanied by depression, are common symptoms of scleroderma. Anemia can also develop with scleroderma, which may contribute to the severity of these symptoms. [verywellhealth.com]

• Asymptomatic

  Depending on the sterility of the retained tissue, it can lead to life threatening surgical complications or may remain asymptomatic for many years and be detected incidentally in imaging studies. It may be mistaken as tumors or aneurysms. [ncbi.nlm.nih.gov]

  Morphea is usually asymptomatic, with occasional itch and rarely pain. Morphea usually begins as a red or purple area of skin that then becomes thickened and white. The thick white areas usually thin out over time and turn brown. [jamanetwork.com]

  These lesions can enlarge when active and then flatten and become asymptomatic with treatment. Deep circumscribed morphea extends into the subcutaneous tissues. [hopkinsmedicine.org]

  Ventricular ectopy, even if asymptomatic, increases the risk of sudden death. Acute renal insufficiency, if untreated, progresses rapidly and causes death within months. [web.archive.org]

• Raynaud Syndrome

  Systemic scleroderma can manifest itself in pulmonary fibrosis, raynaud's syndrome, digestive system telangiectasias, renal hypertension and/or pulmonary hypertension. A chronic multi-system disorder of connective tissue. [icd10data.com]

  People with limited systemic sclerosis have often lived with Raynaud's syndrome for a long time. [sruk.co.uk]

  […] phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia (CREST) syndrome (disorder) Calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia syndrome (disorder) Crest syndrome Lipodermatosclerosis Lung [icd9data.com]

  Systemic scleroderma can manifest itself in pulmonary fibrosis, Raynaud's syndrome, digestive system telangiectasias, renal hypertension and/or pulmonary hypertension. Definition (MSH) A chronic multi-system disorder of CONNECTIVE TISSUE. [fpnotebook.com]

  Puffy fingers in Raynaud's syndrome patients. Differential diagnosis [ 5 ] Several other diseases can present in a similar way to SSc, including: Raynaud's phenomenon from other causes. Vibration injury. [patient.co.uk]

• Fever

  Burt, a tight, noncompliant left ventricle (even with a normal ejection fraction) is vulnerable to stress, such as fever or fluid, which goes back to the lungs as pulmonary edema, causing the patient to become tachycardic. [medscape.com]

  These can include bacteremia, septicemia, fungemia, fever associated with infection, infectious pneumonia, idiopathic pneumonia syndrome, clinical infection (i.e. infection diagnosed with clinical features without identification of an organism) and other [clinicaltrials.gov]

  Fever, loss of appetite, weight loss, and reactive depression were also listed in the constitutional symptoms [30]. [intechopen.com]

  Ronen Brenner, Ilan Ben‐Zvi, Yael Shinar, Irena Liphshitz, Barbara Silverman, Nir Peled, Carmit Levy, Eldad Ben‐Chetrit, Avi Livneh and Shaye Kivity, Familial Mediterranean Fever and Incidence of Cancer, Arthritis & Rheumatology, 70, 1, (127-133), (2017 [doi.org]

  When pulmonary toxicity develops it is characterised by the development of dyspnoea, cough and fever over a period of a few weeks (though more acute and chronic presentations do occur) [ 169 ]. [err.ersjournals.com]

• Cough

  Snapshot A 45-year-old woman presents to her physician with swollen fingers, joint pains, and a dry cough. She reports that this started a year ago and has not improved. She has a past medical history of vitiligo and primary biliary cholangitis. [medbullets.com]

  […] mediated by a vasculitis Unknown etiology Most common form 30-50 Female to male ratio of 3:1 Pulmonary manifestations in 10-25% of patients with scleroderma, but… Lungs are involved in almost 100% of cases at autopsy Clinical Findings Mildly productive cough [learningradiology.com]

  Symptoms may include: Difficulty swallowing Heartburn Bloating Nausea or vomiting Weight loss Diarrhea or constipation If the heart and lungs are affected, you may experience: Shortness of breath Persistent cough Chest pain Kidney problems, such as kidney [ucsfhealth.org]

  […] blue color of hands, feet, or parts of face 0001063 Arthralgia Joint pain 0002829 Arthritis Joint inflammation 0001369 Autoimmunity Autoimmune disease Autoimmune disorder [ more ] 0002960 Cheilitis Inflammation of the lips 0100825 Chest pain 0100749 Cough [rarediseases.info.nih.gov]

  Respiratory/lungs Progressive shortness of breath (dyspnea) and pain in the chest wall (precordial chest pain) and a persistent dry cough. Renal Hypertension or the kidneys not working well. [sclerodermasociety.co.uk]

• Pneumonia

  CLINICAL CASE: A 47-year-old female underwent LDLLT after nine years of symptomatic scleroderma-associated usual interstitial pneumonia and three years awaiting deceased-donor lung transplantation. [ncbi.nlm.nih.gov]

  Treatment options include: Blood pressure medications that dilate blood vessels Immunosuppressant medications to reduce the strength of the immune system Acid relievers to treat acid reflux Flu and pneumonia vaccines Antibiotic treatment for fingertip [utswmed.org]

  Histopathology results from a patient with systemic sclerosis and usual interstitial pneumonia showing patchy interstitial fibrosis in close proximity to unaffected lung tissue. [err.ersjournals.com]

  These can include bacteremia, septicemia, fungemia, fever associated with infection, infectious pneumonia, idiopathic pneumonia syndrome, clinical infection (i.e. infection diagnosed with clinical features without identification of an organism) and other [clinicaltrials.gov]

• Hoarseness

  Other symptoms may include difficulty swallowing, dry cough at night, recurrent chest pain behind the breast bone, persistent hoarseness, asthma (wheezing and shortness of breath), mouth ulcers, and acid taste in the mouth.  [verywellhealth.com]

  […] nose, and throat manifestations Patients may present with the following: Sicca syndrome Poor dentition secondary to sicca syndrome Loosening of dentition caused by alterations in the tooth suspensory ligament and thickening of the periodontal membrane Hoarseness [emedicine.medscape.com]

  Digestive: gastroesophageal reflux disease, bloating, indigestion, loss of appetite, diarrhoea alternating with constipation, sicca syndrome and its complications, loosening of teeth and hoarseness (due to acid reflux). [en.wikipedia.org]

• Sore Throat

  Separately, I went to my GP about a sore throat and he looked through my file. He opened my blood tests and said, “Oh, I see here you’ve got scleroderma”. I had never been told. All he said was, “Oh, it’s an autoimmune condition. [irishtimes.com]

  Normally, our body makes proteins called antibodies to fight infections - for example, when we catch a cold or have a sore throat. These antibodies help to kill the germs causing the infection. [patient.info]

• Constipation

  Most people with progressive GI symptoms do not have scleroderma, but some individuals with early scleroderma may have typical symptoms of irritable bowel syndrome (IBS), like bloating, constipation, or diarrhea, and other GI conditions. [iffgd.org]

  Ask your doctor about medicines for diarrhea, constipation, and heartburn. Lung damage. [niams.nih.gov]

  Mean ± SD scores of UCLA SCTC_GIT 2.0 items were as follows: reflux 1.2 ± 0.8, distention 1.6 ± 1.2, fecal soiling 0.3 ± 0.9, diarrhea 0.8 ± 1, social 1 ± 1, emotional 1 ± 1.1, constipation 0.5 ± 0.9, and total GIT score 0.9 ± 0.6. [ncbi.nlm.nih.gov]

  Symptoms may include: Difficulty swallowing Heartburn Bloating Nausea or vomiting Weight loss Diarrhea or constipation If the heart and lungs are affected, you may experience: Shortness of breath Persistent cough Chest pain Kidney problems, such as kidney [ucsfhealth.org]

  Medicines for diarrhoea and constipation may also be required. When scleroderma affects the lungs, treatment with medicines such as corticosteroids can be helpful. [mydr.com.au]

• Diarrhea

  LES resting pressure had negative correlations with diarrhea score (r = -0.062; p = 0.002). [ncbi.nlm.nih.gov]

  Most people with progressive GI symptoms do not have scleroderma, but some individuals with early scleroderma may have typical symptoms of irritable bowel syndrome (IBS), like bloating, constipation, or diarrhea, and other GI conditions. [iffgd.org]

  Ask your doctor about medicines for diarrhea, constipation, and heartburn. Lung damage. [niams.nih.gov]

  Symptoms may include: Difficulty swallowing Heartburn Bloating Nausea or vomiting Weight loss Diarrhea or constipation If the heart and lungs are affected, you may experience: Shortness of breath Persistent cough Chest pain Kidney problems, such as kidney [ucsfhealth.org]

  Scleroderma of the colon might cause diarrhea and/or constipation. Motor dysfunction of the small intestine can lead to stasis of intestinal contents, and overgrowth of bacteria in the small bowel. [uclahealth.org]

• Abdominal Pain

  Proton Pump Inhibitors Description These medications decrease stomach acid to improve swallowing function and decrease abdominal pain. Physical Therapy Description Physical therapy can improve your flexibility and joint function. [dukehealth.org]

  […] nodule HP:0040281 Feeding difficulties in infancy HP:0040282 Arthralgia HP:0040281 Chondrocalcinosis HP:0040282 Dry skin {has_synonym_type="layperson"} HP:0040281 Gastroesophageal reflux HP:0040281 Malabsorption {has_synonym_type="layperson"} HP:0040282 Abdominal [web.archive.org]

  The symptoms of gastroparesis include early satiety (filling up quickly), nausea, vomiting and abdominal pain. Esophageal dysfunction is a common feature of scleroderma. [uclahealth.org]

  pain Pain in stomach Stomach pain [ more ] 0002027 Abnormal pattern of respiration Abnormal respiratory patterns Unusual breathing patterns [ more ] 0002793 Arrhythmia Abnormal heart rate Heart rhythm disorders Irregular heart beat Irregular heartbeat [rarediseases.info.nih.gov]

  GI symptoms include cramps, bloating, diarrhea or constipation, nausea, abdominal pain, and incontinence. In the lower GI tract, scleroderma can also promote harmful bacteria, slowed movement of food, and reduction of food absorption. [verywellhealth.com]

• Abdominal Bloating

  bloating after meals You may begin to experience spasms of the blood vessels in your fingers and toes. [healthline.com]

  Less often, other parts of your bowel may be affected, leading to symptoms such as: abdominal bloating diarrhoea constipation. [arthritisresearchuk.org]

• Pyrosis

  She had sclerodactyly; tightness of the face skin with thin and shortened lips; telangiectasias on the hands, face, back, and palate; pyrosis; intermittent dysphagia; and ocular and buccal dryness. [ahajournals.org]

• Xerostomia

  Abstract Systemic Scleroderma (SSc) is an autoimmune disease that affects connective tissue, resulting in hardening skin, reduced vascular perfusion, gingival fibrosis, enlarged periodontal ligament, xerostomia, and trigeminal neuralgia. [ncbi.nlm.nih.gov]

  A> </LI> <LI><A HREF="these_body.html#th.0.8.12">9.2 Clinical manifestations</A> <UL> <LI><A HREF="these_body.html#th.0.8.12.8">9.2.1 Microstomia </A> </LI> <LI><A HREF="these_body.html#th.0.8.12.9">9.2.2 Salivary glands, Sj&ouml;gren's syndrome and xerostomia [unige.ch]

  […] incontinence HP:0040282 Peripheral neuropathy HP:0040283 Renal insufficiency HP:0040283 Nephropathy HP:0040282 Cranial nerve paralysis HP:0040282 Behavioral abnormality {has_synonym_type="layperson"} HP:0040283 Decreased nerve conduction velocity HP:0040282 Xerostomia [web.archive.org]

  […] and vomiting 0002017 Restrictive ventilatory defect Stiff lung or chest wall causing decreased lung volume 0002091 Skin ulcer Open skin sore 0200042 Subcutaneous nodule Firm lump under the skin Growth of abnormal tissue under the skin [ more ] 0001482 Xerostomia [rarediseases.info.nih.gov]

• Arthritis

  Estimates of the prevalence of arthritis and selected musculoskeletal disorders in the United States. Arthritis Rheum. May 1998;41(5):778-99. [Medline]. Manetti M, Neumann E, Milia AF, et al. [web.archive.org]

  Arthritis Rheum. 2013 Nov. 65(11):2737-47. [emedicine.medscape.com]

• Hand Stiffness

  Stiffness and swelling Many scleroderma patients have hand stiffness and swelling that is worse in the morning and improves with activity throughout their day. [creakyjoints.org]

  stiffness (81–59%), joint pain (81–64%), and sleep disorders (76–59%). [intechopen.com]

• Swelling of Hand

  Other early symptoms include: Fatigue Swelling of hands and feet Numbness of hands and feet Shiny skin and disappearance of skin folds Ulcers on the fingers Calcium deposits on the fingers Joint inflammation Joints tightening into bent position Muscle [dmso.org]

• Incontinence

  It’s important to have symptoms like diarrhoea and incontinence looked at, to make sure that they don’t have another cause. [versusarthritis.org]

  Incontinence of the bowels. Erection problems (impotence) in men. Heart complications. Several different problems can occur if the muscle of the heart is affected by scar tissue. Lung problems. [patient.info]

  However, it's important to have symptoms like diarrhoea and incontinence looked into to make sure that they don't have another cause. [arthritisresearchuk.org]

  […] has_synonym_type="layperson"} HP:0040281 Gastroesophageal reflux HP:0040281 Malabsorption {has_synonym_type="layperson"} HP:0040282 Abdominal pain {has_synonym_type="layperson"} HP:0040282 Abnormality of the rectum {has_synonym_type="layperson"} HP:0040283 Bowel incontinence [web.archive.org]

  Bowel involvement diarrhea and/or constipation; fecal incontinence Assess for dietary requirements and medication regime. Consult with dietician as necessary. Shortness of breath on exertion Allow patient to set the pace during physical activity. [hospitalnews.com]

• Urinary Incontinence

  Marked cerebral disorders persisted despite adequate rehydration and included agitation, aggressiveness, loss of appetite, insomnia, periods of groaning, compulsive rubbing of the back of her neck on the bed, urinary incontinence, and total indifference [ahajournals.org]

• Seizure

  Both conditions can be associated with neurologic findings, including seizures. We explore a case in which skin findings and seizure burden improved with methotrexate therapy. [ncbi.nlm.nih.gov]

  At age 14, 5 years after anticonvulsants were stopped, she had another generalized seizure (after 8 seizure-free years). A repeat brain MRI was again normal. She has been restarted on anticonvulsants and has been seizure free for the last year. [pediatrics.aappublications.org]

  Seizure 0001250 Skeletal muscle atrophy Muscle degeneration Muscle wasting [ more ] 0003202 Tracheoesophageal fistula 0002575 Showing of 67 | Last updated: 6/1/2019 There is no cure for scleroderma, but treatments are available to relieve symptoms and [rarediseases.info.nih.gov]

  Linear morphea on the head (also called en coup de sabre) may extend inwards to the brain and cause seizures. If morphea crosses a joint, the thickening may limit joint movement. Morphea tends to have a waxing and waning course. [jamanetwork.com]

• Lethargy

  "The lethargy is debilitating and severely limits how much energy is available for a day. "The symptoms may be subtle and slow in onset. They may even just be a cough which does not go away. [bbc.com]

  These include: Fatigue, lethargy Non-specific joint pain and/or inflammation ( arthralgia, arthritis ) Muscle pain Reflux/heartburn Raynaud phenomenon (cold hands with red/white/blue colour changes) Eye dryness, irritation or blurred vision due to ocular [dermnetnz.org]

Classical scleroderma manifests as thickening of the skin, Raynaud’s phenomenon, changes in nail-fold capillaries and anti-nuclear antibodies with a speckled or centromere pattern and nucleolar pattern which though uncommon are specific for scleroderma.

Atypical scleroderma may show any of the above changes without any skin symptoms or only with finger swelling.

Pulmonary function test helps detect early fibrotic changes, alveolitis and pulmonary hypertension. Active lung inflammation can be assessed by bronchoscopy with bronchoalveolar lavage.
HRCT scan is performed to check pulmonary involvement.

Extremity radiography might reveal calcinosis and/or resorption of the distal tufts of fingers and toes.

Echocardiography evaluates pulmonary artery pressure and checks for septal fibrosis or pericardial effusions. If the pulmonary artery pressure is high, then right-heart catheterization is performed to diagnose pulmonary hypertension.

Arrythmias and conduction defects are detected by performing 24-hour ambulatory Holter monitoring.

The gastrointestinal system is evaluated by performing esophago-gastro-duodenoscopy, esophageal manometry and pH monitoring studies.

There is no cure for scleroderma because as of now there is no technique to stop collagen overproduction. One can obtain some measure of relief by various treatments.

Numerous experimental drugs or interventions like interferon-gamma, cyclophosphamide, mycophenolate mofetil, D-penicillamine, photopheresis, allogeneic bone marrow transplantation [21] are used to treat skin thickening. Proteinuria is commonly seen in patients who are receiving D-penicillamine as treatment for scleroderma.

Raynaud’s phenomenon is treated with vasodilators like alpha blockers, calcium channel blockers, local nitrates, angiotensin II receptor inhibitors, serotonin receptor antagonists, statins, etc.

Digital ulcers are treated with phosphodieterase 5 inhibitors or iloprost or with bosentan. Sildenafil is effective and well tolerated by patients with primary Raynaud’s phenomenon and it is also approved to treat pulmonary hypertension [22] [23]. Endothelin receptor antagonists, phosphodiesterase 5 inhibitors and prostanoids are used to treat pulmonary arterial hypertension.

Gatrointestinal symptoms are treated with H2 blockers, proton pump inhibitors, reflux and aspiration precautions, octreotide, prokinetic agents, and laxatives. Patients are advised to take smaller meals.

Angiotensin converting enzyme (ACE) inhibitors and angiotensin II receptor antagonist are used for treating renal diseases.

Immunosuppressants used in the treatment like cyclophosphamide, azathioprine, mycophenolate, methotrexate, intravenous immunoglobulin, sirolimus, alefacept, rituximab, and tyrosine kinase inhibitors like imatinib, dasatinib and nilotinib.
Pruritus can be managed with moisturizers, tricyclic antidepressants (TCAs), histamine 1 (H1) and histamine 2 (H2) blockers, and trazodone.

Arthralgias are controlled with acetaminophen and non-steroidal anti-inflammatory drugs (NSAIDs). Severe flexion contractures may need correction surgery.

In patients with scleroderma, the 5 year survival rate is about 85% while the 10 year survival rate is about 70% [11]. Patients with limited affections will have an approximate 60–70% of 10 year survival rate. In patients with widespread affections, the 10 year survival rate reduces to about 20%. The prognosis is not favourable in patients who are younger at age or suffer from anemia or have a high erythrocyte sedimentation rate (ESR). In cases where there is rapid progress of the disease or widespread extent, the prognosis is not favorable.

Patients with scleroderma can experience complications like pulmonary hypertension, pulmonary fibrosis or renal crisis which can be fatal [2]. Cancer of lungs, liver or bladder and cardiovascular diseases are commonly seen among those suffering from scleroderma [12] [13] [14] [15] [16].

The exact cause of scleroderma is not known [2]. It is considered as an autoimmune disorder [2]. Studies suggest the role of genetic and environmental factors in causing scleroderma. Certain mutations in the HLA gene are said to be related to scleroderma [3] [4]. Environmental factors like silica, aromatic and chlorinated solvents, benzene, ketones, epoxy resins, radiations are factors known to contribute to increasing scleroderma [3] [4] [5] [6] [7].

Certain pathologic mechanisms like fibroblast activation, cellular and humoral immunologic derangement and endothelial cell injury are involved. Drugs like bleomycin and pentazocine may contribute to scleroderma.

Scleroderma is found to affect women more than men, the ratio being 4 to 9: 1 [2]. It manifests mainly between the age group of 20 to 50 years, though it can affect any age group. Asians are less affected comparatively [2] [8] [9].

The incidence is about 10 times higher among African Americans as compared to Native Americans. In the United States, the incidence is 19 cases per million population annually, and the prevalence is approximately 240 cases per million population.

Scleroderma affects many organ systems but mainly the skin, the gastrointestinal tract, respiratory, cardiovascular, renal and genitourinary systems and the vasculature. The pathophysiology of scleroderma involves alterations at the level of vasculature like endothelial cell damage and apoptosis causing vascular leakiness presenting initially as tissue oedema.

Anti-endothelin cell antibodies cause impaired angiogenesis and impaired vasculogenesis which increases the vascular damage.
Cytokines and growth factors help generate myofibroblasts from fibroblasts. Studies of patients with scleroderma show presence of dysregulated transforming growth factor β in fibroblasts and myo-fibroblasts. These lead to increased collagen and other protein deposition causing fibrosis. Extracellular matrix production is increased by IL-6 and TGF-β produced by B cells. Endothelin signalling has also been implicated in the pathophysiology of the fibrosis [10].

Patients suffering from scleroderma are advised to maintain the core body temperature to minimize chances of Raynaud’s phenomenon. They must avoid large doses of vitamin C as it accelerates collagen formations and deposition. Skin wounds caused by ischemic lesions or calcinosis must be protected from contamination.

In order to avoid contractures or minimise them, one must undergo regular physiotherapy. Nicotine worsens scleroderma and hence, patients must stop smoking. Patients must avoid exposure to cold weather to prevent any circulatory problems. In cases of gastrointestinal tract symptoms, one must eat small but frequent meals.

Scleroderma is a word of Greek origin. ‘Skleros’ means hard or indurated and ‘derma’ means skin. It was originally defined by Hippocrates as thickened skin [1]. In 1945, Robert Goetz described it as progressive systemic sclerosis to indicate its systemic affections and defined it as a gradually progressing disease.

Scleroderma or systemic sclerosis is characterized by skin induration and thickening along with tissue fibrosis and chronic inflammatory penetration in various visceral organs, fibro-proliferative vasculopathy, and humoral and cellular immune alterations.

Systemic sclerosis is a multi-organ, chronic disease that affects almost all systems. It mainly affects the skin causing tightening and hardening of the skin. It also involves the gastrointestinal tract, the respiratory system, kidneys, the heart, genitourinary systems and the vasculature. It also causes pain, swelling and contractures of the musculoskeletal system.

It is supposed to be an autoimmune disorder where one’s own immune system harms the tissues of the body. Genetic and environmental factors are also considered to play a role in the same.

For patients with minimal spread, the 10-year survival rates are 60-70% and for those with widespread disease, the 10-year survival rates are only 20%.

At the level of the skin, it causes fingers to turn blue or white in cold temperature called as Raynaud’s phenomenon, thickening and hardening of the skin, ulcers on the tips of fingers and toes and facial mask-like look. It causes joint pains, stiffness, weakness of muscles, and contractures. It also affects the lungs causing breathlessness and high blood pressure in the lungs. In the gastric system it causes acid reflux, constipation or diarrhoea. It can lead to kidney damage and failure. It also causes affections of the nervous system.

There is no specific treatment. Treatment is aimed at controlling the symptoms and preventing further complications. Medicines normally used are immunosuppressants, corticosteroids, NSAIDs, Antacids, Anti-hypertensives etc. Surgeries may be required for contractures. Patients must avoid smoking as nicotine worsens scleroderma. To avoid raynaud’s phenomenon one must avoid exposure to cold. Wounds or injuries must be protected from contamination.

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