【Overview】.
Nodal disease (sarcodosis) is a multisystem disease of unknown cause, with clinical manifestations of hilar lymph node enlargement, pulmonary infiltrates and skin and eye lesions, and pathology characterized by non-caseating granulomas of the involved tissues.
Overview of diagnostic points
Diagnosis can be confirmed based on symptoms, signs and/or suspicious lesions on chest X-ray, combined with relevant examinations and histopathological examination.
Clinical manifestations
1, non-specific manifestations include fatigue, weakness, loss of appetite, weight loss, fever and excessive sweating.
2.Pulmonary nodulopathy has significant chest X-ray changes with mild clinical symptoms, and there is a clear pattern of evolution of the lesion: initially there are enlarged hilar/mediastinal lymph nodes, and then they gradually shrink but the interstitial lesions in the lungs become obvious; and later the enlarged hilar/mediastinal lymph nodes completely disappear, leaving only the interstitial lesions in the lungs.
3.Extrapulmonary nodular disease
(1) Granulomatous and vascular lesions of the eye most often manifest as uveitis, which can have ocular discomfort, photophobia and lacrimation, and can lead to glaucoma, cataract and blindness.
(2) Heart: Myocardial involvement may include conduction disturbances and arrhythmias, with common rhythm changes such as sinus syndrome, sinus bradycardia, atrioventricular block and frequent pre-term contractions, which are important causes of sudden death from nodal disease.
(3) Neurological nodal disease includes central and peripheral, the former has a worse prognosis and is also an important cause of death in nodal disease. The former has a poor prognosis and is also an important cause of death in nodal disease. It occurs in the mesencephalon and can present with increased cranial pressure with impaired consciousness, headache, convulsions, etc. A variety of focal symptoms, mainly numbness, can also be seen.
(4) Hepatic nodular disease: The main manifestations are hepatomegaly and abnormal liver function (mainly elevated ALP and bilirubin), and severe patients may show cirrhosis-like manifestations.
(5) Rheumatic manifestations of nodular disease
Arthritis: Acute arthritis is the most common rheumatic manifestation of nodular disease, which is symmetrical, wandering polyarthritis, often involving knee, ankle, PIP, wrist and elbow joints, mainly manifesting as joint pain and limitation of movement, lasting 2-3 weeks, and leaving no sequelae after subsiding. Chronic arthritis is rare and persistent arthritis can be associated with joint destruction and loss of function.
Bone damage: Commonly seen in the bones of the hands and feet, but also in the skull, vertebrae and long bones, mostly asymptomatic, associated with chronic skin lesions, when extensive invasion of the hands, can form diffuse spongy thickening, advanced or extensive lesions with osteolytic changes, and even cause pathological fractures.
Muscle damage: muscle lesions are divided into myopathic and myxomatous types. The former has reduced muscle strength and muscle atrophy in the proximal extremities, and CPK may be normal or elevated, while the latter may have multiple, variable-sized nodules palpable, and may have pressure pain and tumor spasm.
Cutaneous nodular disease: there may be nodular erythema, lupus frostbite, scar infiltration, lupus-like erythroderma, subcutaneous lesions and skin atrophy.
(6) Other system manifestations: there may be upper respiratory tract involvement such as nose, pharynx and larynx, with corresponding nasal congestion, hoarseness and dyspnea; there may also be parotid, digestive tract and kidney involvement.
4, nodular disease can be combined with tuberculosis, tumor, RA and amyloidosis.
Laboratory tests
1, serum angiotensin-converting enzyme (SACE) activity is increased, serum lysozyme and β2-microglobulin can be elevated some patients have increased blood uric acid, in the active phase of the disease may have leukopenia, anemia, ESR fast, IgG increased and transient hypercalcemia and hypercalciuria.
2, Pulmonary function is predominantly restrictive changes, and obstructive disease may also be present.
3, Bronchoalveolar lavage fluid examination shows an increased lymphocyte ratio, increased Th/Ts ratio and IgG/albumin <1.
Imaging examination
Chest X-ray changes are divided into 4 stages.
Stage 0: normal chest X-ray performance.
Stage I: Bilateral hilar and/or lymph node enlargement, often accompanied by paratracheal lymph node enlargement.
Stage II: Bilateral hilar lymph node enlargement with pulmonary infiltrates and extensive symmetrical distribution of pulmonary lesions bilaterally, with nodular, punctate or flocculent shadows, along with gradual regression of hilar shadows.
Stage III: Only pulmonary infiltrates or fibrosis were seen without hilar lymph node enlargement.
Other tests
1.Nodular disease antigen (Kvein) test The lymph nodes or spleen tissue of patients with acute nodular disease were made into 1:10 saline suspension as antigen for intradermal injection, and the formation of granuloma or histological examination showing granuloma was positive after 4~6 weeks of observation. Because there is no standardized antigen, the application of this experiment is limited.
2.Tuberculin test Most of them show no or very weak reaction.
3. Skin nodules, intramuscular nodules and other affected histopathological examinations show epithelioid granulomas without caseous changes.
Differential diagnosis
It should be differentiated from tuberculosis of hilar lymph nodes, lymphoma, hilar metastatic carcinoma and granuloma due to non-nodular disease.
Treatment overview
Most patients with this disease have a good prognosis. Patients with symptoms and life-threatening complications can be treated with corticosteroids and immunosuppressive agents such as tumor cotinine, azathioprine and methotrexate.