Systemic Sclerosis, is it curable?: Digital Ulcer, Everything

Definition:



Digital Ulcer


Systemic sclerosis is a connective tissue autoimmune disease characterized by widespread small vessel obliterative disease and fibrosis of the skin and multiple internal organs, including the heart, kidneys, and gastrointestinal tract. The diagnosis is clinical, but laboratory tests can support the diagnosis and contribute to the prognosis. Specific treatment is difficult, and the focus is usually on the treatment of complications.


Classification
  1. Limited systemic sclerosis (CREST syndrome)
  2. Systemic systemic sclerosis (diffuse skin involvement)
  3. Systemic sclerosis sinus scleroderma

In limited systemic sclerosis (CREST syndrome-skin calcification, Raynaud’s phenomenon, esophageal dyskinesia, sclerosis, telangiectasia), the patient’s face, elbows, and the distal skin of the knees are tight, and may also suffer Gastroesophageal reflux disease. This type is characterized by slow progress and often complicated by pulmonary hypertension.

In systemic sclerosis with diffuse skin involvement, patients will experience Raynaud’s phenomenon and gastrointestinal (GI) complications. This type usually develops rapidly. The main complications are Interstitial lung disease and renal crises

In systemic sclerosis, patients with sinus sclerosis have antibodies related to systemic sclerosis and visceral manifestations of the disease, but no skin tightness.



Systemic sclerosis symptoms

Skin symptoms
  1. In the skin, the main signs are, systemic sclerosis causes hardening and scarring of the skin
  2. Werner's disease ( it is one type of progeria, looking older than he is )
  3. Chronic hypostatic edema (chronic pulmonary edema, heart failure)
  4. The skin swelling is usually symmetrical and gradually hardens
  5. The skin may look tight, red, or scaly. Vessels of body surface may also be more visible
  6. The pigmentation of the skin is lost. Hypo or hyperpigmentation in almost all cases
  7. Telangiectasia may develop

Visceral disease
  1. Idiopathic pulmonary fibrosis
  2. Rheumatoid arthritis
  3. Sarcoidosis
  4. Infiltrative cardiomyopathies
  5. Intestinal obstruction

There are two forms of systemic sclerosis:
Limited (80%) and diffuse (20%). In limited, we found CREST syndrome, skin changes limited to face and hands. CREST syndrome patients have a much better prognosis. In diffuse-type skin, chances are the more diffuse involving trunk, extremities, and tendon friction rubs are found in the wrist, ankles, and knees.



What is the causation or Pathophysiology?

The pathophysiology includes vascular harm and enactment of fibroblasts; collagen and other extracellular proteins in different tissues are overproduced.

In this disease, the skin grows more conservative collagen filaments in the reticular dermis, epidermal diminishing, loss of rete stakes (epithelial expansions that undertaking into the hidden connective tissue), and decay of dermal limbs. Immune system microorganisms may amass, and broad fibrosis in the dermal and subcutaneous layers creates. In the nail folds, slim circles enlarge and some microvascular circles are lost. In the limits, ongoing aggravation and fibrosis of the synovial layer and surfaces and periarticular delicate tissues happen.

Esophageal motility gets weakened, and the lower esophageal sphincter gets clumsy; gastroesophageal reflux and optional injuries can create. The intestinal muscularized mucosa degenerates, prompting pseudodiverticula in the colon and ileum. Interstitial and peribranchial fibrosis or intimal hyperplasia of little pneumonic conduits can create; if long-standing, aspiratory hypertension can result. Diffuse myocardial fibrosis or cardiovascular conduction anomalies happen. Intimal hyperplasia of interlobular and arcuate supply routes can create inside the kidneys, causing renal ischemia and hypertension.

Fundamental sclerosis fluctuates in seriousness and movement, going from summed up skin thickening with quickly reformist and regularly lethal instinctive association (diffuse foundational sclerosis) to confined skin contribution (frequently the fingers and face) and moderate movement (regularly quite a few years) before instinctive sickness creates.


What is Digital Ulcer?
This ulcer situated particularly on fingertips and less ordinarily the knuckles—are normal.
Digital ulcers (DUs) are a typical noticeable sign of the reformist vascular infection that portrays the
Systemic Sclerosis illness measure. It does not just affect essentially patients' personal satisfaction and handwork, but at the same time are a biomarker of inward organ inclusion and of sickness seriousness. The etiology of (advanced) vascular sickness in Systemic Sclerosis is multifactorial, and large numbers of these elements are possibly agreeable to remedial mediation.




Difference between scleroderma and systemic sclerosis?
Scleroderma is related to skin and organs of the body, forms scar on fibrous tissue, whereas Systemic Sclerosis affects the internal organ like the lung, kidney, liver, gastrointestinal tract, etc.



Is systemic sclerosis a disability?
Yes, it is considered a physical disability. Because in this disease, the fibrous tissue terns into scar tissue and that's why causes the hardening of external as well as internal organs. The patient can not perform actions as a normal person.



Is systemic sclerosis curable?
It is sorry to say, that there is no or little possibility of curing this disease. Because every protecting fibrous tissue transfers into deathly rigid scar tissue, which is not flexible or chance of granulation formation to recover. It is considered an auto-immune disease. In "Constitutional treatment" can have a little light but the chance is very poor.



Can systemic sclerosis affect the eyes?
Definitely!, the surrounding of eyes turns into rigidity. Lower and upper palpable skin will be rigid, organ of vision may have irreversible changes.



Is Systemic Sclerosis affected Digestive Tract?
Diffuse scleroderma can influence any piece of the gastrointestinal tract. The most widely recognized indication in the throat is reflux esophagitis, which might be convoluted by esophageal injuries or generous narrowing of the esophagus. This is best at first treated with proton siphon inhibitors for corrosive suppression, yet may require bougie dilatation on account of stricture. Scleroderma can diminish motility anyplace in the gastrointestinal tract.
Esophageal brokenness is the most continuous instinctive aggravation and happens in many patients. Dysphagia (typically retrosternal) as a rule grows first. Indigestion can cause acid reflux and injury. Barrett's throat happens in 30% of patients and inclines to complexities (eg, adenocarcinoma). Hypomotility of the little gut causes bacterial abundance that can prompt malabsorption. Air may enter the harmed inside divider and be noticeable on x-ray (pneumatosis intestinalis). Spillage of gut substance into the peritoneal pit can cause peritonitis. Unmistakable wide-mouthed diverticula can create in the colon. Biliary cirrhosis may create in patients with restricted foundational sclerosis (CREST disorder).


Multiple Sclerosis and Systemic Sclerosis
In Multiple sclerosis, their organs can be involved except primary effect on nerve tissue. In Systemic sclerosis, is poorly described, probably affects entire internal body organs including nerve tissue.



How to Diagnose?
Clinical approach: Symptoms of Raynaud's phenomenon, distal skin thickening, and visceral organ involved.
Pulmonary arterial hypertension or interstitial lung disease might be reported

Laboratory findings
Anemia
Erythrocyte sedimentation rate(ESR) often is elevated
Urinalysis abnormalities (such as proteinuria, RBC, and casts) reflect renal involvement



Raynaud's phenomenon
  1. Raynaud's disease
  2. Occupational trauma
  3. Shoulder-hand syndrome
  4. Vascular (including SLE and polymyositis)
  5. Hematologic abnormalities
  6. Polycythemia vera
  7. Cryoglobulinemia
  8. Vinyl chloride toxicity


Is systemic sclerosis serious?
Yes, Systemic sclerosis is a serious auto-immune disorder. It is occupied whole-body skin as well as organs and mostly affects women and children. It is almost incurable.


Treatment
  1. Education: Patient education should focus on information about specific organ involvement and the tendency for spontaneous fluctuation in disease activity to occur.
  2. Monitoring organ involvement
  3. Barium swallow and esophageal manometry
  4. Pulmonary function testing
  5. Routine urinalyses and 24 hours urine
  6. No smoking
  7. Small frequent meals with proper chewing
  8. Avoid tea, coffee



Homeopathic medicines
Homeopathy believes in constitutional treatment after proper case-taking,
Some medicines can help to little bit such as
Carbo vegetabilis 30, Heloderma, Secale cor.

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