Nodular disease is an endogammatory disease with multisystem and multiorgan invasion. It often invades the lungs, bilateral hilar lymph nodes, and more than 90% have pulmonary changes; followed by skin and ocular lesions; superficial lymph nodes, liver, spleen, kidney, bone marrow, nervous system, heart and almost every organ of the body can be involved. The disease is a self-limiting disease with a mostly good prognosis and a tendency to remit spontaneously. Nodular disease is mostly seen in middle-aged and young people, with a higher incidence in cold, cold and mock heat areas.
Clinical manifestations The main symptoms are.
1, respiratory symptoms cough, coughing a small amount of sputum, or a small amount of hemoptysis; may have weakness, fever, night sweats, loss of appetite, physical reasoning reduction, etc.. When the lesion is extensive, chest tightness, shortness of breath, and even cyanosis may occur, which may be aggravated by combined infection, emphysema, bronchiectasis, and pulmonary heart disease.
2.Neurological symptoms Invasion of the central nervous system, brain nerve damage, convulsions, paralysis and intracranial hypertension may occur.
The most common manifestation of skin is erythema nodosum. Eye damage may include eye pain, blurred vision, ciliary body congestion, etc. Some patients have enlarged liver and spleen.
Treatment
As most patients can be in remission, those with stable disease and no symptoms do not need treatment. For those with obvious symptoms, prednisone 30-60 mg/day can be used in divided doses and gradually reduced to 15-30 mg/day after 4 weeks, with a maintenance dose of 5-10 mg/day for 1 year or longer. Its up can be treated with chloroquine, methotrexate, azathioprine, etc. Drugs that can cause the increase of blood calcium and urine calcium are prohibited.
Sarcoidosis is a granulomatous disease with multi-system and multi-organ involvement. It often invades the lungs and bilateral hilar lymph nodes, and clinically more than 90% have pulmonary changes, followed by skin and eye lesions, and almost every organ of the body can be involved. It is a self-limiting disease with mostly good prognosis and a tendency to spontaneous remission.
X-ray staging
Nodular disease is most likely to invade intra-thoracic organs and tissues, especially hilar lymph nodes and lungs. Abnormal chest X-ray is often the first finding of nodular disease, and about 90% of patients have chest X-ray changes. The staging of nodular disease on plain radiographs is still not uniform. 1961, Scanddin8 divided nodular disease into four stages (stages 1-4), and in recent years, it has been divided into five stages (stages 0, 1-4).
Stage 0 Negative lung x-ray with clear lungs.
Stage I Enlarged hilar and/or mediastinal lymph nodes on both sides, often accompanied by enlarged right paratracheal lymph nodes, accounting for about 51% of cases.
Stage II Enlarged hilar lymph nodes with pulmonary infiltrates. The pulmonary lesions are widely and symmetrically distributed on both sides in the form of nodular, punctate or flocculent shadows of 1-3 mm. In a few cases, they may be distributed on one lung or in certain lung segments. The lesions may gradually resorb over a year or develop into interstitial lung fibrosis in about 25% of cases.
Stage III Only pulmonary infiltration or fibrosis without hilar lymph node enlargement is seen in about 15% of cases.
The presentation of the above stages does not indicate the order of progression of nodular disease.
The National Conference on Pulmonary Nodular Disease (revised draft, 1988) decided to divide the chest X-ray manifestations of nodular disease into three stages
Stage I: Bilateral enlargement of hilar and mediastinal lymph nodes. This stage accounts for about 43% of cases. 95% of cases are bilateral, symmetrical and clearly defined on both sides. 5% of cases are unilateral. The prognosis of this stage is good, and most of them can resolve on their own. 80% resolve within one year, 10% resolve within the second year, and l0% become chronic. The older the age of onset, the more likely it is to become chronic.
Stage II: Bilateral hilar and mediastinal lymph node enlargement with pulmonary cornular, fibrous nodular or cotton ball shadows. This stage accounts for about 41% of cases, and the pulmonary lesions may appear simultaneously with or during the regression of the enlarged lymph nodes. The lung shadows resolve spontaneously in 60% of cases, with 50% disappearing within one year, 30% within two years, and 20% within 3-7 years. Most of the lesions in both lungs show diffuse nodular, corn-like, patchy or reticulonodular shadows.
Stage III: The lungs show fibrotic changes, which account for about 16% of this stage. The lung lesions are diffusely distributed, and the X-ray manifestation is dominated by reticulonodular-like structures, complicated by pulmonary maculopathy and cystic bronchiectasis, most of which are the formation of interstitial fibrosis in the lung.
In addition, pleural nodular disease is extremely rare, accounting for about 0.8% of cases, but recently it has been reported to be up to 10%, mostly coexisting with pulmonary lesions, with exudate and predominantly lymphocytes, most of which resolve spontaneously within 4-8 weeks.
In addition, increased serum immunoglobulins, hypercalcemia, hypercalcemia, and increased alkaline phosphatase are also useful for diagnosis.
Sarcoidosis is a granulomatous disease with multi-system and multi-organ involvement. It often invades the lungs and bilateral hilar lymph nodes, and clinically more than 90% have pulmonary changes, followed by skin and eye lesions, and almost every organ of the body can be involved. It is a self-limiting disease with mostly good prognosis and a tendency to spontaneous remission.
Diagnosis of nodular disease
In addition to history and X-ray, the diagnosis of nodular disease is mainly based on tissue biopsy or Kveim’s skin test.
1.Tissue biopsy.
(1) First, take material from superficial parts, such as lymph nodes, rash, subcutaneous nodes, etc. If negative, take lymph nodes of the fat pad of the anterior oblique muscle.
(2) Take bronchial mucosa biopsy under fiberoptic bronchoscopy and take lung tissue biopsy through the bronchial wall (TBLB), which can mostly obtain positive results, especially for patients with stage II and III chest X-ray, with a positive rate of 63%-97%.
2, Kveim’s test.
The lymph nodes or spleen of patients with active nodular disease are taken as antigen and made into saline suspension, injected intradermally into the forearm with 0.1-0.2 ml, and the skin is excised after 4-8 weeks, and pathological biopsy is performed, which is positive if non-caseous epithelioid nodules are shown. The positivity rate is about 65%-92%. The principle of this test is considered by most to be related to cellular immunity and may be a type of delayed hypersensitivity reaction.
3. Nodulin reaction.
Cellular immunity is low in nodular disease, which is manifested by a negative or weakly positive nodulin reaction (5 units of old nodulin). This method can be one of the auxiliary diagnostic methods.
4, Angiotensin Converting Enzyme (ACE).
Angiotensin I is converted into angiotensin II with elevated pressure by ACE in pulmonary capillary endothelial cells.Libermann’s found that ACE in blood increases during the active phase of nodular disease, which is helpful for the diagnosis of nodular disease, especially the determination of lesion activity status.
5. Cytological examination of bronchoalveolar lavage (BAL) fluid.
It is believed that BAL cytology is more sensitive than ACE for the diagnosis of early alveolitis in nodular disease and the estimation of activity. It shows an increase in total cell count; an increase in lymphocytes (7% of total cells in normal individuals, up to 50% in patients with nodular disease); an increase in T lymphocytes, which can account for more than 28% of total cells; and an increase in the helper T lymphocyte/suppressor T lymphocyte ratio, which can increase from 1.8/1 to l0.5/1.
6. 67 gallium (67Ga) scan.
67Ga mostly accumulates in the metabolically active and proliferative regions of inflammatory granulomas, which may be related to the lysozyme binding of neutrophils to visualize. The increase of 67Ga index in patients with nodular disease is positively correlated with the total number of BAL lymphocytes and T lymphocytes, which is quite helpful for the diagnosis of the intensity of alveolitis, and is also an indicator of the activity of nodular disease.
7. Measurement of metalloendopeptidase activity.
The activity of this enzyme was three times higher in patients with nodular disease than in controls. The activity of the enzyme was 2 times higher in the active phase of nodular disease than in the inactive phase. And 4 times higher than normal controls. The activity of this enzyme was not significantly different from controls in cases of active tuberculosis, bronchopulmonary carcinoma, and idiopathic interstitial lung fibrosis. This indicates that the measurement of this enzyme is useful for the diagnosis of nodular disease and its active phase.
In addition, increased serum immunoglobulin, hypercalcemia, hypercalcemia, and increased alkaline phosphatase are also helpful in the diagnosis.
Sarcoidosis is a granulomatous disease with multi-system and multi-organ involvement. It often affects the lungs and bilateral hilar lymph nodes, with clinical changes in more than 90% of the lungs, followed by skin and eye lesions, and almost every organ of the body can be involved. It is a self-limiting disease with mostly good prognosis and a tendency to spontaneous remission.