Mixed connective tissue disease is a group of conditions that exhibit a combination of symptoms from lupus erythematosus, dermatomyositis, scleroderma, or other connective tissue disorders. It is characterized by high titers of ENA antibodies in the blood, particularly a high positive rate of anti-ribonucleoprotein antibodies. Renal involvement is rare, and the disease responds well to corticosteroids, resulting in a favorable prognosis.

bubble_chart Clinical Manifestations

1. Systemic lupus erythematosus-like manifestations, including butterfly-shaped rash on the face, scarring discoid lupus erythematosus or subacute cutaneous lupus erythematosus-like rash, arthritis, alopecia areata, photosensitivity, etc.
2. Systemic scleroderma-like manifestations, with Raynaud's phenomenon in 85% of cases, appearing early, accompanied by finger swelling, sclerosis, tapering of fingertips resembling sausages, and slowed esophageal motility.
3. Dermatomyositis-like manifestations, featuring purplish-red edematous patches on the eyelids, purplish-red papules and atrophic patches on the dorsal aspects of the finger joints, proximal muscle weakness, and myalgia.
4. The heart, lungs, liver, spleen, and central nervous system may be involved, with possible systemic lack of strength and fever.
5. High-titer anti-nRNP antibodies (characteristic), speckled pattern of antinuclear antibodies with high titers, elevated serum creatine kinase, anemia, leukopenia, hypergammaglobulinemia, and accelerated erythrocyte sedimentation rate.

bubble_chart Diagnosis

1. Clinically, there are mixed symptoms of systemic lupus erythematosus, dermatomyositis, and systemic scleroderma, making it difficult to definitively diagnose which specific disease is present.
2. Raynaud's phenomenon is evident in the early stages, with hard swelling of the fingers resembling sausages, along with facial edema, tight skin, thickening, and poor elasticity.
3. The presence of high-titer anti-nRNP antibodies and speckled fluorescent antinuclear antibodies, along with high titers, can confirm the diagnosis.

bubble_chart Treatment Measures

Principles of Treatment

1. General treatment;
2. Vasodilators, anti-inflammatory drugs, skin-softening medications;
3. Symptomatic treatment;
4. Supportive therapy.

1. For mild cases, corticosteroids within the scope of "A," Root Leaf or Flower of Common Threewingnut, compound formula Salvia tablets, enhanced warmth, avoiding cold, overexertion, and supportive therapy may be used;
2. For cases complicated by lung, heart, brain, or gastrointestinal issues, colchicine, increased hormone dosage, immunomodulatory drugs, and options within the scope of "B" and "C" may be selected;
3. Previously, the prognosis of this disease was considered favorable, but recent findings indicate a mortality rate of 4–7%. Death may result from severe complications such as pulmonary stirred pulse hypertension, renal insufficiency, myocarditis, myocardial infarction, heart failure, colon perforation, cerebral embolism, or generalized vasculitis.

bubble_chart Cure Criteria

1. Cure: Symptoms disappear, skin lesions subside, laboratory tests return to normal;
2. Improvement: Most symptoms disappear, skin lesions subside by more than 50%, laboratory tests tend to normalize;
3. No cure: Symptoms do not completely disappear, skin lesions subside <50%，實驗室檢查改善，或未全恢復正常。