Undifferentiated connective tissue disease (in)

  Clinical presentation Overview The disease often starts insidiously The average time from the onset of clinical symptoms to consultation is 2 to 3 years, with an average of 38 months. The clinical manifestations of the patient’s health search are often mild, and nonspecific symptoms such as malaise, low-grade fever, and enlarged lymph nodes are common. Some larger clinical investigations found that the most frequent symptoms were arthralgias, Raynaud’s phenomenon and skin mucosal damage, while involvement of important organs such as the kidneys and central nervous system was rare. During the follow-up, it was found that the clinical symptoms of patients could fluctuate with the course of the disease and treatment, mostly showing a gradual remission trend, but overall the disease activity did not change much.   Skin lesions 1. Skin mucosal lesions. Skin lesions are quite common, and the manifestations of the rash type are diverse, with some patients having a rash as the first symptom.  Discoid erythema is more common than in SLE patients, appearing in about 34% of patients. It can be found in all races and has a higher incidence in black patients. It presents as a red papule above the skin surface on exposed areas of the body, with the head and neck being the most common health search. The rash varies in size and shape, and the surface is often scaly, leaving scars and local skin atrophy after healing.  The incidence of zygomatic erythema is about 10% for red papules on the cheeks, which can have a typical butterfly-like distribution, or irregular shape.  The incidence of photosensitivity is between 13% and 24%. Dry mouth and dry eyes occur in about 18% of patients. The incidence of mucosal ulcers is lower than that of SLE, ranging from 3% to 13%. Diffuse swelling of both hands and subcutaneous nodules have also been reported.  Joint and muscle lesions 2. This lesion is more common. About 37% to 80% of patients may present with arthralgia or arthritis, with an average incidence of 55% and an average incidence of 42% for arthritis. It is mostly non-invasive polyarthritis and rarely occurs in those with joint destruction and deformity. It can involve all joints of all sizes, including interphalangeal joints, metatarsophalangeal joints, and mandibular joints, but large arthritis is more common. Morning stiffness may be present, but is usually short-lived. Synovial fluid examination is mostly suggestive of inflammatory exudate, with low cell count, yellow color when protein content is high, and negative bacterial culture.  Muscle involvement is mostly seen as myalgia and muscle weakness in the proximal muscle groups of the extremities. Individual reports may even show mild to moderate elevation of myoenzymes but no abnormal EMG or mild myogenic damage, no significant abnormalities on muscle biopsy, not meeting the diagnostic criteria for myositis or other connective tissue diseases.  Vasculitis Raynaud’s phenomenon 3, vasculitis Raynaud’s phenomenon is one of the most common health search clinical manifestations of UCTD seen in about 50% of patients, and may persist for many years as the only clinical symptom manifested as episodes of pallor, bruising and flushing of the extremities, with local pain or numbness before the onset of more cold or emotional agitation and other triggers, a few minutes or tens of minutes after the gradual relief of small artery spasm is its pathological basis. Long-term frequent attacks may show local soft tissue atrophy and necrosis and other dystrophic manifestations, and in severe cases, bone resorption of the extremity.  In addition, about 5% of patients may develop hypertension, and imaging examination of local vascular wall hyperplasia or stenosis important organ vasculitis such as cardiac vasculitis, renal artery stenosis, arteriovenous embolism, etc.,.  Pulmonary and cardiac lesions 4. Plasmacytitis is the most common, but the incidence is slightly lower than that of SLE, about 11%, and may manifest as pleural effusion, pericardial effusion or both. The severity of the disease varies from no obvious clinical manifestations in mild cases to cardiac tamponade in severe cases. Plasmapheresis is often suggestive of leaking fluid and may be positive for antinuclear antibodies.  Other pulmonary manifestations include interstitial fibrosis and interstitial pneumonia, and other rare manifestations such as endogenous lipoid pneumonia. Interstitial pulmonary fibrosis is insidious and presents with progressive dyspnea. X-ray examination reveals thickened lung texture and disorganized pulmonary function suggesting reduced diffusion. Exercise lung function and high-resolution CT of the chest are more sensitive and can assist in early diagnosis.  Cardiac lesions can involve the whole heart, including pericarditis, myocarditis and endocarditis, etc. Clinical symptoms such as chest tightness, palpitations and dyspnea, ECG can have various arrhythmias and ST-T changes, etc.  Hematologic lesions 5. About 20% of patients have this lesion can be manifested as leukocytes, thrombocytopenia and anemia to moderate reduction in leukocytes and non-hemolytic anemia is the most common health search. Thrombocytopenia is present in about 7% of patients and can be quite severe. Individual cases have a pronounced bleeding tendency and even cause death. Hemolytic anemia is rare, mostly chronic disease anemia and iron deficiency anemia. Whole blood cytopenia is also seen.  Renal damage 6. The incidence of renal damage is around 11% and is less common in patients with discoid erythema and ANA negativity. Clinical manifestations may include edema, hypertensive proteinuria, hematuria and elevated serum creatinine levels, but rarely result in severe renal insufficiency.  Other neurological impairments 7. Other neurological impairments are rare and may manifest as psychiatric symptoms such as migraine, convulsive behavior abnormalities and hallucinations or organic neurological disease manifestations such as peripheral neuritis, headache, hemianopia, sensory and mobility disorders.  Complications 1, Raynaud’s phenomenon long-term frequent occurrence may appear local soft tissue atrophy necrosis and other dystrophic manifestations, severe cases of limb bone resorption about 5% of patients can appear hypertension, but also important organ vasculitis such as cardiac vasculitis, renal artery stenosis arteriovenous embolism, etc..  2.Pleural effusion, pericardial effusion or both are concurrent.  3.Individual cases have obvious bleeding tendency and even cause death.