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    Home > Active Ingredient News > Immunology News > Young people in the blooming season have difficulty walking and have acne on their faces. Beware of this rheumatism!

    Young people in the blooming season have difficulty walking and have acne on their faces. Beware of this rheumatism!

    • Last Update: 2021-03-25
    • Source: Internet
    • Author: User
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    *Only for medical professionals to read and refer to rare rheumatism and immune diseases, you can earn it when you see it! 1 The wheelchair was pushed into the open clinic door of a young man in blooming season and was slowly pushed open.
    A young man in blooming season came to the rheumatology clinic in a wheelchair because he was tortured by pain in his left hip for a whole year.

    Xiao Wang is a freshman from a prestigious university, but he is full of sadness in front of him, and he can't see the youthful vigor.

    One year ago, he had pain in his left hip without any cause.

    Obviously after the initial activity, there will be rest and activities in the future.

    One month ago, the above symptoms worsened, and low back pain and left hip pain occurred, which affected walking and restricted lumbar spine movement.
    So he went to the local hospital and found HLA-B27 negative, erythrocyte sedimentation rate (ESR) and C-reactive protein ( CRP) was elevated, MRI of the sacroiliac joint revealed bone marrow edema of the left iliac bone (Figure 1).
    The first hospital considered to be diagnosed with ankylosing spondylitis (AS), and celecoxib combined with methotrexate and leflunomide Symptoms were not significantly relieved after Mitte treatment.

    Gradually appear low-grade fever, Tmax 37.
    8℃, no obvious fluctuations in body temperature, no chills, chills, no night sweats, no cough, sputum, frequent urination, urgency, abdominal pain, diarrhea, etc.
    , deny skin and family history, deny digestive system disease History, denying family history of ocular diseases such as uveitis.

    No hobbies such as smoking or drinking.

     Figure 1.
    MRI of the patient's sacroiliac joint showed bone marrow edema of the left iliac bone (pointed by the arrow).
    2 A doctor with a sharp eye, without letting go of any details, faced such a patient with poor treatment effect and severe disease affecting normal function, rheumatism The immunologist performed a comprehensive physical examination on Xiao Wang.
    The results were as follows: stable vital signs, clear mind, good nutrition, wheelchair pushed in, unable to stand on his own, poor mood, unwilling to communicate, and answered relevant questions. Acne-like skin rashes can be seen on the cheeks of the face, which are fused into pieces, and some pus spots are present (Figure 2).
    The anterior tibial skin of the lower limbs can be scattered with red punctate papules, and some are fused into pieces, with central necrosis, and 3 bedsores in the sacrococcyx.
    Skin rash, crusted on the surface.

    The rest of the skin and mucous membranes are not as rash, yellowish or ulcers.

    Examination of the heart, lungs, and abdomen was no exception.

    Mild tenderness of the right sternoclavicular joint, tenderness of the left hip, limited internal rotation of the left hip, positive left 4-character sign, and no difference in physical examination of the right hip.

    There is no tenderness in the lumbar spine, and the mobility test cannot be completed because the patient cannot stand.

    Each joint ST-.

    After the rheumatic immunologist completed a detailed physical examination, there was a lot of doubt in his mind: HLA-B27 negative AS? "Although young people in the blooming season can have more serious acne, the rash of the lower limbs cannot be explained by acne.
    Is the decubitus-like rash in the sacrococcygeal area really caused by restricted movement of the left hip?" So the doctor asked the medical history and learned that Xiao Wang 5 Years ago, repeated facial and back acne appeared, and it has worsened in the past six months, especially the symmetrical distribution on the face, with occasional ulceration and discharge.
    I have been treated with fosfomycin, metronidazole, minocycline and isotretinoin.
    Bad.

    With doubts, the doctor prescribed a comprehensive laboratory and imaging examination for Xiao Wang.
    The results showed that his inflammation indicators were elevated.
    The imaging revealed left sacroiliitis, and chest CT revealed erosive destruction of the right sternoclavicular joint (figure).
    3) The bone scan showed that there were radioactive concentrated areas in the bilateral sternoclavicular joints and sacroiliac joints, and inflammatory changes were considered (Figure 4).

    The patient’s blood, urine and stool routine, liver and kidney function, electrolytes, coagulation function, immunoglobulin, and complement were all normal, and immune related tests [rheumatoid factor (RF), ANA, ENA spectrum, anti-double-stranded DNA (dsDNA) , Anti-neutrophil cytoplasmic antibodies (ANCA), HLA-B27] are all negative, and infection-related indicators (hepatitis B, hepatitis C, HIV, T-SPOT) are all negative.

    Blood culture and abscess fluid culture were negative.

     Figure 2.
    The patient’s facial acne.
    Figure 3.
    The patient’s chest CT showed erosive destruction of the right sternoclavicular joint (pointed by the arrow).
    Figure 4.
    The patient’s bone scan showed that the bilateral sternoclavicular and sacroiliac joints showed radioactive concentration areas (arrows).
    Referred to) 3 Sacroiliac arthritis + skin rash + bone destruction, find a breakthrough in diagnosis According to the doctor's detailed understanding and evaluation of Xiao Wang's medical history, the case is summarized as follows: • Onset of disease: young male, chronic disease, progressive worsening.

    •Clinical manifestations: Acne-like rash + pain in the left buttocks affects walking.

    • Auxiliary examination: increased inflammation indicators, bone destruction of the right sternoclavicular joint, and sacroiliitis on the left.

    • Physical examination: facial and trunk rash, limited movement of the left sacroiliac joint.

    • Past history, personal history, family history: no special.

    In this way, Xiao Wang's complicated condition can be summarized as: sacroiliitis + skin rash + bone destruction.

    Rheumatologists began to make differential diagnosis with sacroiliitis as the breakthrough point.

    Xiao Wang’s sacroiliac arthritis is not the same as the common AS patients.
    First, he is a unilateral sacroiliac arthritis; second, it has a serious impact on the patient’s function (inability to walk, resulting in bedsores).
    Treatment with non-steroidal anti-inflammatory drugs (NSAIDs) is ineffective; in the end, the accompanying severe rash cannot be explained by AS.

    So besides AS, what other diseases can cause sacroiliitis? The answer includes infectious diseases, neoplastic diseases, and other autoimmune diseases.

    Among them, infectious diseases and neoplastic diseases are usually unilateral sacroiliitis, while AS and other autoimmune diseases can be unilateral or bilateral.

    Common pathogens of infectious sacroiliitis include: Staphylococcus aureus (IVDA), pneumococcus, Klebsiella, Proteus, Pseudomonas aeruginosa, Brucella, Mycobacterium and fungi.
    Mycobacterial infection is usually The manifestations of bacteremia (high fever, chills, etc.
    ) are not obvious and require special attention, but this case is negative for T-SPOT and negative blood culture.
    There is no more evidence to support the infection. Tumor diseases need to actively search for the basis of the tumor.
    This patient has bone destruction at the sternoclavicular joint, and more attention should be paid to the tumor.
    However, the bone scan results indicate that the bone destruction may be inflammatory and the patient’s age is not the age of high incidence of tumors.
    , So it is still necessary to further search for the cause.

    Other autoimmune diseases that can cause sacroiliac arthritis include Behcet's disease, juvenile arthritis, and SAPHO syndrome, in addition to spondyloarthritis.

    SAPHO syndrome is a group of rare inflammatory diseases of bones, joints, and skin.
    Its typical clinical manifestations are Synovitis, Acne, Pustulosis, Hyperostosis and Bone The first letters of Osteitis form the name of the syndrome.

    The cause of the disease is not yet known, and it usually occurs in people aged 30-50.

    The manifestations of bones and joints are its hallmark symptoms, manifested as osteomyelitis, synovitis, bone hypertrophy, and sacroiliitis, often involving the anterior chest wall bones (65%-90%) and other axial bones and joints (32% -52%).
    Peripheral joint involvement is rare (<30%), and can be manifested as osteomyelitis, bone destruction, and bone hypertrophy on imaging.

    Skin manifestations are common in more than 60% of patients, manifesting as palmoplantar pustulosis, severe acne, or pustular psoriasis.

    Because the disease is rare, there is no evidence-based medical evidence or consensus guidelines for its treatment.
    Symptomatic treatment is often based on the main symptoms of the patient, including NSAIDs to relieve pain, intra-articular injection or oral glucocorticoid anti-inflammatory, and disease-improving anti-rheumatic drugs (DMARDs) , Such as methotrexate, sulfasalazine, cyclosporine A, leflunomide, thalidomide, etc.
    ) as second-line treatment, antibiotics (such as doxycycline) to treat acne, also for patients with poor response Biological agents (such as tumor necrosis factor antagonists) can be used for treatment.

    Xiao Wangyou has tried NSAIDs and DMARDs in the previous treatment, but the effect was not good.
    Therefore, the rheumatologist chose the biological agent inaercept for him.
    Two weeks after the treatment, Xiao Wang's left hip pain was significantly relieved and he was able to resume walking.
    After one month of treatment, the facial rash has also improved significantly (Figure 5), both ESR and CRP have returned to normal, and the young teenager finally showed a long-lost smile.  Figure 5.
    The patient's treatment of posterior acne significantly improved.
    Reference materials: [1]Cianci F, Zoli A, Gremese E, et al.
    Clinical heterogeneity of SAPHO syndrome: challenging diagnose and treatment.
    Clin Rheumatol 2017; 36:2151.
    [2] Olivieri I, Padula A, Palazzi C.
    Pharmacological management of SAPHO syndrome.
    Expert Opin Investig Drugs 2006; 15:1229.
    [3]Firinu D, Murgia G, Lorrai MM, et al.
    Biological treatments for SAPHO syndrome: an update.
    Inflamm Allergy Drug Targets 2014; 13:199.
    Professor Guo Qiang Spondyloarthritis (SpA) is a common disease in rheumatology and immunology, including two types of axial and peripheral.
    Among them, AS is the most familiar and typical of axial spondyloarthritis Represents the type of disease.

    With the development of disease susceptibility gene screening and the development of MRI technology that is conducive to early diagnosis, AS diagnosis has made significant progress in recent years.

    However, some new questions still need to be answered.
    Is HLA-27(+) all AS? Can the bone marrow inflammation signal found by MRI be caused by other reasons besides AS? How is the AS of HLA-B27(-) diagnosed? What differential diagnosis is needed for MRI atypical? From the initial suspicion of AS to the final diagnosis of SAPHO syndrome, this patient did not neglect to think about the latter two issues.

    The incidence of SAPHO syndrome is less than 1/100,000, and it is not often encountered.
    The treatment is also like AS.
    Most of them are more effective for tumor necrosis factor inhibitors (TNFi).
    After the patients are used, arthritis and skin lesions can be resolved.

    However, these do not mean that SAPHO can be confused with AS.

    From different susceptibility genes to obviously different clinical manifestations, they all indicate that they are two different diseases with different pathogenesis mechanisms.

    Understand this point, when the TNFi treatment fails, we can also have other targeted drug options for the treatment of SAPHO, such as interleukin-1 (IL-1), IL-17, and IL-23 antagonists that are involved in the pathogenesis of impetigo.
    , Or even a JAK inhibitor with a wide range of effects.

    Of course, being able to identify SAPHO, a rare rheumatic immune disease, simply based on the characteristic joint + skin lesions, the diagnosis process itself is very attractive and it is worth learning.

    Expert profileProfessor Guo Qiang, Deputy Executive Director, Department of Rheumatology and Immunology, Renji Hospital, Shanghai Jiaotong Medical College, Standing Committee Member and Secretary of the Immunoadsorption Academic Committee of the Chinese Medical Doctor Association Member and Secretary of the Rheumatology Sample Bank of the Chinese Medical Biotechnology Association Member and Secretary of the Rheumatology Branch of the Shanghai Medical Association Member of the Clinical Immunization Branch of Shanghai Immunology Association Member of the Expert Committee of Shanghai Rheumatology Clinical Quality Control Center
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