Systemic scleroderma: diagnosis and medical treatment

2021-07-24 11:48 PM

Systemic sclerosis, as a heterogeneous disease, is reflected by a wide range of organ involvement, and the severity of the disease.

The term scleroderma is used to describe the presence of thick, hard skin. Scleroderma is the hallmark of systemic sclerosis.

Systemic sclerosis is a chronic multisystem disease characterized by widespread vascular dysfunction and progressive fibrosis of the skin and internal organs. The diagnosis of systemic sclerosis and related disorders is based primarily on the presence of characteristic clinical findings and is supported by specific serological abnormalities.

Systemic sclerosis is a heterogeneous disease, reflected by a wide range of organs involved, disease severity, and outcome.

Systemic scleroderma is a chronic autoimmune disease of unknown etiology, clinically characterized by thickening and hardening of the skin due to collagen accumulation, involving multiple organ systems including the gastrointestinal tract, heart, lungs, kidneys and blood vessels.

Clinical symptoms

Skin: skin thickening, edema, calcification, skin pigmentation disorder.

Joints: arthritis, swelling, heat, redness, deformity, ...

Extremity examination: cyanosis, pallor or necrosis of the extremities (Raynaud's syndrome).

Lungs: dry cough, crackles, wet rales, coagulation syndrome, ...

Cardiovascular: there may be hypertension, arrhythmia, pericardial rub, heart murmurs, signs of right heart failure (hepatomegaly, central venous insufficiency,...).

Digestive: choking, digestive disorders, reflux syndrome.

Kidney: manifestations of glomerulonephritis, nephrotic syndrome, renal failure (edema, oliguria, red urine, oliguria, anuria).

Subclinical symptoms

Complete blood count: there may be decreased red blood cells, decreased hemoglobin, increased white blood cells, increased erythrocyte sedimentation rate.

Quantitative urea increased, creatinine increased, blood potassium increased in case of renal failure.

Total urinalysis: proteinuria, erythrocytes, erythrocytes, and casts of urine in case of kidney damage.

Serum protein electrophoresis: albumin decreased, globulin increased.

Pulmonary function measurement: FVC, FE V 1.

Chest X-ray, CT scan of the chest (if interstitial lung injury is suspected).

Dopper ultrasound of extremities, renal vessels: narrowing of vessels, decreased flow rate in arteries and veins.

ECG.

Gastroduodenal endoscopy.

Antinuclear antibody (ANA) is specifically positive for scleroderma.

1980 American College of Rheumatology Diagnostic Criteria

Main standard

Scleroderma: thickening of the skin, symmetrical hardening of the skin on the fingers, the base of the interphalangeal or knuckle joints.

Skin lesions may involve the entire hand, neck, chest, abdomen, and face.

Sub-Criteria

Scleroderma of the extremities: skin lesions described above limited to the fingers.

Concave scars or loss of stroma in fingers and toes: consequences of vascular occlusion or infarction.

Bibasal fibrosis: grid or nodular opacity, honeycomb or glass opacities at the base of the lungs on straight or CT thoracic film

Diagnosis is confirmed when there is 1 major criterion or > 2 minor criteria.

Differential diagnosis

Rheumatoid arthritis

Inflammation, swelling, heat, red pain in many joints, especially the joints of fingers and fingers.

Morning stiffness.

Rheumatoid factor is positive in approximately 80% of patients.

Diagnosis is confirmed when there are more than 4/7 criteria in the table of rheumatoid arthritis diagnostic criteria of the American Rheumatology Association in 1987.

Systemic lupus erythematosus

Erythema butterfly-shaped face, arthralgia, alopecia.

Antinuclear antibody test, anti-ds-DNA positive.

Diagnosis is confirmed when ằ 4/11 criteria of the American College of Rheumatology in 1997.

Dermatitis, polymyositis

Myalgia, weakness mainly in the proximal extremities, may be accompanied by erythematous patches on the skin.

Blood tests for muscle enzymes are elevated.

There is usually a malignancy in > 50% of cases.

The principles of treatment

Treatment of disease control combined with symptomatic treatment.

Treatments

Disease control drugs

Glucocorticoid:

Methylprednisolone, prednisolone or prednisone 1.5-2mg/kg/24 hours, gradually reduce dose 10mg/week and maintain at dose 5-10mg/day.

Little effect in disease control and many side effects should be avoided for prolonged use.

Cyclophosphamide:

Indications: interstitial lung lesions of advanced systemic scleroderma unresponsive to other drugs.

Dosage: 1-2mg/kg/24 hours or intravenous infusion every 3-4 weeks, each time 500-1000mg, reduce the dose when there is renal failure. Duration of treatment > 6 months.

Treatment monitoring: Blood count once a week, liver and kidney function test before treatment and once a month during treatment. Stop treatment if SLBC < 1.5 G/L, TC < 100 G/L, urinary HC (+). Maximize fluid resuscitation during treatment to increase drug elimination.

Cyclosporin A:

Indications: interstitial lung lesions of advanced systemic scleroderma.

Dosage: 2 - 5mg/kg/24 hours, taken in 2 divided doses for > 6 months.

Treatment monitoring: weekly BP measurement. Test renal function before treatment and once a month, glomerular filtration rate every 3 months during treatment.

Symptomatic treatment

Raynaud's syndrome:

Avoid exposure to cigarette smoke, avoid cold, keep the whole body warm, especially the hands and avoid psychological trauma.

Drugs: Oral calcium channel blockers (especially nifedipine) are most effective. If ineffective, intravenous iloprost, oral prazosin or topical nitroglycerin may be used.

When there is ulceration of the extremities: ensure sterility, avoid superinfection of the injured area. Surgical removal of necrotic tissue and amputation are the last resort.

Skin damage:

Limit bathing because it can dry out the skin.

Use a moisturizer containing lanolin.

Colchicine may be effective for symptomatic subcutaneous calcification.

Musculoskeletal symptoms:

Nonsteroidal anti-inflammatory drugs (NSAIDs) are effective in most cases.

In case of myositis or tendinitis - bursitis unresponsive to NSAIDs, low dose glucocorticoids (prednisone 10 - 20 mg/day) can be used. need to coordinate rehabilitation.

If glucocorticoids are not effective, methotrexate can be added.

Digestive symptoms:

Patients with esophageal motility disorders should eat many small meals during the day and lie down with their heads elevated after meals, avoiding eating at night. Consider adding antacids (eg, cimetidine, omeprazole).

Cases of abdominal distention, diarrhea, weight loss, and malabsorption due to small bowel motility disorders need to be treated with broad-spectrum antibiotics such as trimethoprim-sulfamethoxazole, metronidazole, or ciprofloxacin every 2 weeks, need vitamin supplements and mineral salts.

Cardiopulmonary symptoms:

Interstitial pneumonia: early-stage should be treated with glucocorticoids and immunosuppressive drugs (especially cyclophosphamide). In the final stage, when the fibrous tissue grows much, consider lung transplantation.

Pulmonary hypertension alone: ​​continuous oxygen, use of anticoagulation and treatment of right heart failure can improve symptoms well. Consider intravenous iloprost.

Manifestations of pericardial effusion, heart failure, and arrhythmias are treated similarly to these in other conditions.

Kidney damage:

Use diuretics if you have kidney failure.

If severe renal failure is unresponsive to diuretics, early dialysis or renal transplantation should be considered.

In the presence of hypertension, antihypertensive treatment with calcium channel blockers, angiotensin II inhibitors or ACE inhibitors (if there is no renal artery stenosis or severe renal impairment).