1 case, 2 tables, the clinical manifestations of sarcoidosis have been clarified!

*Only for medical professionals to read for reference to improve the clinical understanding of sarcoidosis! Sarcoidosis is a systemic disease with non-caseating granuloma as its pathological characteristics.
Clinically, it mainly involves involvement of the lungs and mediastinal lymph nodes.
It can also involve the skin, eyes, gastrointestinal tract, nervous system, kidneys, and spleen.
, heart, skeletal muscle
.

Sarcoidosis is not uncommon in the clinic, but it is easy to be misdiagnosed and mistreated.
This is because the clinical manifestations of patients with sarcoidosis are very heterogeneous, ranging from no obvious clinical symptoms to progressive disease, which can lead to decreased lung function.
Or some patients died due to progressive pulmonary fibrosis or cardiac involvement, including sudden cardiac death (arrhythmia) or congestive heart failure (myocarditis)
.

The author recently read a case report and literature review of sarcoidosis with arthritis as the first symptom published by Pang Mengduan, Department of Rheumatology and Immunology, the First Affiliated Hospital of Dalian Medical University, published in the Chinese Journal of Clinical Immunity and Allergy
.

Sarcoidosis is relatively rare in which arthritis is the main symptom.
I will share this case with you here, combined with the recommendations for the diagnosis and treatment of sarcoidosis, in order to improve the clinical understanding of sarcoidosis
.

Case data: The patient, male, 32 years old
.

He was admitted to this hospital as "Arthritis pending investigation" on March 25, 2020 due to "swelling and pain of both ankles and back swelling of both feet for 1 month"
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The patient experienced swelling and pain in both ankles, swelling of the back of the feet, and mild restriction of movement 1 month ago without any predisposing factors.
There was no fever, cough, chest tightness, skin rash and other symptoms
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Ultrasound from the outside hospital showed that the arteries and veins of both lower extremities were normal; the metatarsophalangeal joints of the right foot were normal; the right ankle joint was effusion, and the subcutaneous soft tissue around the right ankle joint was edema and thickened (the left foot and left ankle joint were not examined)
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Physical examination after admission: no superficial lymphadenopathy
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Breath sounds in both lungs were clear, and no rales were heard
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Both the cardiac examination and abdominal examination showed no abnormalities
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Both ankles and backs of the feet are obviously swollen, symmetrical, mild tenderness, and mild activity limitation
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Auxiliary examination: blood test showed white blood cells 9.
8×109/L, hemoglobin 150g/L, platelets 338×109/L; liver and kidney function normal, blood calcium 2.
27mmol/L; ANA 1:100+, anti-ds-DNA antibody, anti ENA antibody, anti-CCP antibody, and anti-neutrophil cytoplasmic antibody were all negative
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Rheumatoid factor, immunoglobulin, complement, and IgG4 were normal
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C-reactive protein (CRP) 11.
7 mg/L, erythrocyte sedimentation rate (ESR) 12 mm/h
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PPD test, Mycoplasma pneumoniae IgG, IgM antibody, G test, GM test, serum Epstein-Barr virus PCR, serum cytomegalovirus PCR were negative
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Blood gas analysis is normal
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Chest HRCT showed multiple hilar hilums, multiple enlarged lymph nodes in the mediastinum, thickened pleura on both sides, multiple pleomorphic lesions in both lungs (Figure 1 A, B)
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Ultrasonic bronchoscopy-transbronchial needle aspiration biopsy (EBUS-TB-NA) mediastinal lymph node histopathology: a large number of epithelioid cells formed nodules and scattered inflammatory cell infiltration under light microscope, suggesting epithelioid granulomatous lesions, not clear Tumor cells (Figure 2)
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Alveolar lavage fluid acid-fast bacilli smear and PCR, atypical pneumonia pathogen PCR, bacterial smear and culture, fungal smear and culture were all negative
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Combined with the patient’s clinical symptoms and signs, as well as related laboratory examinations, imaging examinations, and pathological examinations, the diagnosis was confirmed as sarcoidosis, and methylprednisolone 40 mg qd was given oral treatment
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The patient's joint swelling and pain relieved after a few days
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Three months later (June 2020), the chest CT showed obvious absorption of the lesion (Figure 1C, D)
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Nine months later (December 2020), methylprednisolone was stopped
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After 14 months (May 2021), the patient's condition was still under control, and neither lung disease nor joint disease had recurred
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Figure 1 Chest HRCTA and B of patients with sarcoidosis: HRCT showed both hilar and multiple enlarged lymph nodes in the mediastinum (arrow), thickened bilateral pleura, and multiple pleomorphic lesions in both lungs (2020-03-30); C, D: Reexamination of chest HRCT showed obvious lesion absorption (2020-06-15) Figure 2 EBUS-TBNA mediastinal lymph node puncture tissue in patients with sarcoidosis showed epithelioid granulomatous lesions (2020-04-01), the arrow pointed to epithelioid Cells, Epithelioid Cells Form Nodules Discussion 01 Sarcoidosis Overview Since being recognized about 130 years ago, sarcoidosis has been a multi-system disease of unknown etiology characterized by non-caseating granuloma tissue infiltration
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The disease occurs all over the world, affecting people of all races and all ages
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Approximately 70% of patients are between 25 and 45 years old
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In Europe and Japan, the second peak of the incidence occurred in women over 50 years of age
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The prevalence of the disease is about 4.
7 to 64 per 100,000 people, and the incidence is 1.
0 to 35.
5 per 100,000 people
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Among them, Northern Europeans and African Americans have the highest incidence, and Japanese have the lowest incidence
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The ratio of male to female is about 1.
20:1.
75
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02 Clinical manifestations The clinical manifestations of patients with sarcoidosis are very heterogeneous, ranging from no obvious clinical symptoms to related clinical manifestations caused by the progressive aggravation and recurrence of the disease
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Table 1 summarizes the clinical characteristics, physical examination manifestations, imaging manifestations and laboratory examination results for the diagnosis of sarcoidosis
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Table 1 Clinical features that support the diagnosis of sarcoidosis.
Clinical features highly suspected of sarcoidosis.
Clinical manifestations of sarcoidosis.
Löfgren syndrome a.
7 Effective for experimental treatment of cranial nerve palsy.
Renal failure effective for experimental treatment of cardiomyopathy or AVNB Unexplained spontaneous/inducible VT signs frostbite-like rash, uveitis, optic neuritis, nodular erythema, red or violet macules, papules, subcutaneous nodules, scleritis, retinitis, lacrimal gland enlargement, direct laryngoscopy, granulomatous lesions, symmetrical parotid glands Enlarged liver/splenomegaly imaging manifestations of hilar lymphadenopathy (X-ray chest X-ray, CT, PET) peripheral pulmonary lymphatic nodules (CT) Gadolinium-enhanced MRI suggests enhanced (central nervous system) osteolysis Sexual changes, bone cystic degeneration/puncture-like lesions, trabecular bone lesions with increased uptake of the parotid glands (Ga imaging or PET) Mainly distributed in both upper lungs or diffuse lesions in both lungs (X-ray chest X-ray, CT, PET) bronchus Bundle thickening (CT) two or more intrathoracic lymphadenopathy (CT, MRI, PET) cardiac inflammatory lesions (MRI, PET, Ga imaging) liver/spleen enlargement, or multiple nodules (CT, PET, MRI) bone inflammatory lesions (Ga imaging, PET, MRI) unexplained LVEF decrease (cardiac color Doppler ultrasound, MRI) other detection of hypercalcemia or hypercalcemia, accompanied by abnormal vitamin D metabolism, elevated bACEc Kidney stones (calcite), vitamin D metabolism is not detected.
BALF is lymphocyte-based or the ratio of CD4/CD8 is increased by 3 times of alkaline phosphatase.
The first episode, Ⅲ° atrioventricular block in young and middle-aged patients above the normal limit Stasis: ACE: angiotensin converting enzyme; AVNB: atrioventricular node block; LVEF: left ventricular ejection fraction; MRI: nuclear magnetic resonance; PET: positron tomography; VT: ventricular bradycardia; aLöfgren's The clinical manifestation of the syndrome is symmetrical hilar lymphadenopathy with nodular erythema or periarthritis; abnormal b-vitamin D metabolism is normal or low, 1.
25-dihydroxyvitamin D is normal or elevated, and 25 ‐Hydroxyvitamin D is normal or decreased; elevated cACE means that the ACE level is higher than 1.
5 times the upper limit of normal.
In sarcoidosis, arthritis is a rare clinical symptom, with a prevalence of 6% to 22%, which can be divided For acute arthritis and chronic arthritis
.

Acute arthritis is mainly affected by the ankle joint (about 90%).
It can also involve knee joints, wrist joints, elbow joints, and hand joints.
It is manifested as joint pain, swelling, joint effusion and swelling of surrounding soft tissues.
Arthritis (2 to 4 joints) (about 88%)
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Acute arthritis mostly manifests as mild non-specific inflammation dominated by monocytes without granuloma infiltration, and rarely causes joint deformities
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At present, there is no unified diagnostic criteria for sarcoidosis acute arthritis.
In 2002, Visser et al.
proposed the diagnostic criteria for sarcoidosis acute arthritis based on previous case data: symmetric biannular arthritis; duration of less than 2 months; age younger than 40 years old; erythema nodosa
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The above four criteria can be diagnosed when three criteria are met (must include symmetric biannular arthritis), with a sensitivity of 93% and a specificity of 99%
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This patient is in line with the diagnosis of sarcoidosis with acute arthritis
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Chronic arthritis is manifested by granulomatous inflammation invading the joints and surrounding tissues.
Symmetric oligoarthritis is the main clinical manifestation, which mostly involves large and middle joints
.

Joint manifestations are diverse, such as mucosal bursitis, tenosynovitis, sacroiliitis, carpal tunnel syndrome, Jaccoud disease, etc.
, and often combine with other system diseases, especially skin diseases
.

Chronic arthritis is very rare, and the course lasts for a long time, leading to joint deformities
.

Sarcoidosis with arthritis as the main symptom or the only symptom is rarely reported, which deserves clinical attention
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03 Pathological manifestations Histopathological manifestations are necessary for the diagnosis of most sarcoidosis; the characteristic manifestations are compact, well-differentiated granulomas, the central area is surrounded by multi-layered immune cells surrounding the core area of ​​macrophages, Multiple megakaryocytes, loose lymphocytes in the peripheral area (mainly T cells, B cells in a small number of patients), and occasionally a few dendritic cells
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The vast majority of sarcoidosis is non-necrotizing granulomas, but a small number of patients with sarcoidosis (especially those with nodular pulmonary sarcoidosis) can present both necrotizing and non-necrotizing granulomas
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The differential diagnosis of granulomatous disease has a wide range, as shown in Table 2
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It is worth noting that although in general, sarcoid granulomas have some histopathological characteristics that are different from other granulomatous diseases, sarcoidosis cannot be diagnosed by histopathological manifestations alone, such as chronic beryllium.
Disease and sarcoidosis are very similar in pathology
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Table 2 Histopathological characteristics of sarcoidosis.
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