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Suffering from mild scleroderma. Treated with methotrexate. showing symptoms of fibromyalgia. Diagnosed with polyneuritis

My wife is suffering from mild scleroderma, now the skin of face and neck have become very supple.
she was treated with Methotrexate for 2 weeks.Now she has fibromyalgia symptoms with neuritic pain left leg and anaesthesia in left foot. Sural nerve biopsy was done which showed polyneuritis.She cant walk , becomes breathless.She has reflux oesophagitis, proved by endoscopy. Angiogram was done it is normal.She is on pregabalin 75 mg BD. She has mild pedal oedema. She has sleeplessness also.It is bizarre of symptoms.I will be glad if you can throw some light
Asked On : Wed, 17 Apr 2013
Answers:  2 Views:  315
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Neurologist 's  Response

Scleroderma is a chronic systemic autoimmune disease (primarily of the skin -"derma") characterized by fibrosis (or hardening -"sclero"), vascular alterations, and autoantibodies. There are two major forms:

Limited systemic sclerosis/scleroderma involves cutaneous manifestations that mainly affect the hands, arms, and face. It was previously called CREST syndrome in reference to the following complications: Calcinosis, Raynaud's phenomenon, Esophageal dysfunction, Sclerodactyly, and Telangiectasias. Additionally, pulmonary arterial hypertension may occur in up to one-third of patients and is the most serious complication for this form of scleroderma.

Diffuse systemic sclerosis/scleroderma is rapidly progressing and affects a large area of the skin and one or more internal organs, frequently the kidneys, esophagus, heart, and lungs. This form of scleroderma can be quite disabling. There are no treatments for scleroderma itself, but individual organ system complications are treated.

Immunosuppressants like steroids methotrexate ,mycept, azathioprine are used to suppress the immune mechanism .

Since the disease also cause vasculitis of small vessels supplying nerves hence they can also have poly neuritis which in turn produce pedal edema

Continue with Pregabalin and along with it she also requires methylcobal supplements

The prognosis is generally good for limited cutaneous scleroderma patients who escape pulmonary complications, but is worse for those with the diffuse cutaneous disease, particularly in older age, and for males.
Dr Sandhya Manorenj
Neurologist Hi tech city Hyderabad

Answered: Sat, 4 May 2013
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Internal Medicine Specialist Dr. Omer's  Response
SSc is categorized into diffuse cutaneous SSc (dcSSc) and limited cutaneous SSc (lcSSc) subsets on the basis of the extent and distribution of skin involvement. LcSSc is commonly associated with the CREST syndrome.
Skin involvement is a nearly universal feature of SSc, and is characterized by variable thickening and hardening of the skin. The fingers, hands, and face are generally the earliest areas of the body involved. Edematous swelling and erythema of the skin generally precedes skin induration. Other skin manifestations can occur. Malaise, fatigue, arthralgias and myalgias are common.

The most obvious clinical manifestation of vascular dysfunction of SSc is Raynaud phenomenon, which is due to arterial vasoconstriction in the digits. Characteristic sequential color changes in the digits of pallor ("white"), acrocyanosis ("blue"), and reperfusion hyperemia ("red") are precipitated by cold, stress, or even change in temperatures.

Vascular injury and subsequent chronic damage underlies other serious complications of SSc, including pulmonary artery hypertension, scleroderma renal crisis and gastric antral vascular ectasia, and also contributes to the pathogenesis of cardiac and gastrointestinal complications.

Gastrointestinal involvement is present in most patients and can involve any part of the gastrointestinal tract. Symptoms are present in more than half of patients and most commonly result from chronic gastroesophageal reflux, with subsequent chronic esophagitis and stricture formation, Barrett's esophagus, and abnormal motility.
Pulmonary involvement is seen in more than 70 percent of patients. The principal pulmonary manifestations are interstitial lung disease and pulmonary vascular disease. The latter leads to pulmonary arterial hypertension and is more common in patients with limited cutaneous disease.
Clinical renal disease is observed in up to half of patients. Findings may include some degree of proteinuria, a mild elevation in the plasma creatinine concentration, and/or hypertension. Pathologic vascular changes are present in the kidneys in 60 to 80 percent of patients with dcSSc. Scleroderma renal crisis occurs in up to 10 to 15 percent of patients, generally among those with dcSSc.
Cardiac disease in SSc may be primary or secondary, and is associated with a poor prognosis. Musculoskeletal disease, neuromuscular and genitourinary involvement may also occur, and the risk of malignancy may be increased
Answered: Wed, 17 Apr 2013
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