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*For medical professionals only
During the diagnosis process, be sure to keep a clear head~
Synopsis:The young man of the patient thought it was just ankylosing spondylitis (AS), but he did not expect an unexpected discovery to gain a lot! Let's follow in the footsteps of Attending Physician Wang Lifang of Peking University Shougang Hospital (Interpreting Physician) and Chief Physician Li Ru (Commenting Physician) of Peking University People's Hospital to learn about this case together!
Case introduction
.
Complaints: 12 years of intermittent low back pain, swelling and pain in the left elbow joint for 2 months, aggravated for 12 days
.
"Admitted to hospital
on 12 March 2019.
Anamnesis: 1 year
with a history of gout.
Denial of a history of
drug allergy.
Family history: the mother has recurrent low back pain, which is not systematically diagnosed and treated
.
There is no difference in
personal history/marriage and childbearing history.
Current medical history: 12 years ago,
the patient developed low back pain without obvious cause and was diagnosed in the orthopedic department
.
Perfecting the spine x-ray shows lumbar joint bulging
.
The symptoms are recurrent, untreated, and can improve
on their own.
Before 2 months, the patient developed swelling and pain in the left elbow joint without cause, and the patient did not care
.
12 days ago, the patient had low back pain again after exercising, and he was treated in the emergency department of our hospital, and the improvement of chest and lumbar X-rays showed that the thoracolumbar segment of the spine was consistent with AS changes
.
Today, for further diagnosis and treatment, the patient was admitted
to the emergency department as "ankylosing spondylitis".
Physical examination: T 36.
2 °C; P 70 times/min; R 20 times/min; BP 108/68mmHg； Pure spirit, spiritual; The heart boundary is not large, the heart rate is 70 bpm, rhythm is aligned; The thoracic cage is symmetrical, the breathing sounds of both lungs are clear, and no dry and wet rales are heard; Soft abdomen, no tenderness, rebound tenderness, liver and spleen are not reached
.
Specialist examination: Schober test 0.
5cm, chest expansion 1cm, occipital distance 1.
5cm, finger distance 42cm, positive 4 character test
.
Auxiliary examination:
- Blood, urine, stool routine, biochemistry, coagulation, thyroid function, bone metabolism, tumor markers, hepatitis B, blood transfusion, tuberculosis are normal;
- negative for autoantibodies;
- Erythrocyte sedimentation rate is normal;
- C-reactive protein 19.
13mg/L; - Interleukin 6: 8.
6pg/ml
. - HLA-B27 positive;
- ECG: sinus rhythm; Mild ST segment changes;
- Non-contrast chest CT scan showed no abnormalities;
- Plain CT scan of sacral joints: the space between the two sacral joints disappeared, the sacral joints fused, and the bilateral sacral joints were consistent with AS changes;
- Bone density: osteoporosis
.
The patient is young male, inflammatory low back pain, HLA-B27 positive, sacroiliac joint CT shows sacroiliitis, lumbar spine rays show bamboo-like changes, according to the 1984 revised AS New York standard and the 2009 ASAS development of the axial SpA classification standard, AS diagnosis is certain!
Figure 1: 1984AS New York Standard and 2009 ASAS Central Axis SpA classification standard
In addition to AS, there is a "real murderer"
But an unexpected discovery made everyone wonder, a 3cm x 3cm red and swollen mass can be seen on the inside of the flexor side of the patient's left elbow joint, tenderness is weak and positive, the boundary is clear, there is a sense of flexibility, there is a slight soreness, and the patient usually does not care
.
According to monism, this is a peripheral manifestation of AS involvement, but is the truth really that simple?
The patient further examined the elbow CT and surprisingly did not see joint space narrowing, ligament changes, or joint fusion
.
CT results show multiple bone destruction areas and soft tissue density foci next to the left elbow joint and in the bone, which is obviously not an imaging manifestation of AS, is it gout?
Fig.
2: CT results of the left elbow joint were further examined for magnetic resonance, and the results
were even more surprising, showing multiple low-intensity lesions next to the left elbow joint and in the bone, and multiple sac-like bone destruction areas
of different sizes.
It seems that the swelling and pain of the left elbow joint is not caused by AS and gout, what is the cause?
Figure 3: Left elbow MRI results
Learn 8 differential diagnoses together
1.
Ganglion cyst: It is a cystic mass that occurs in the tendon sheath of the joint, which is caused by the degeneration of connective tissue in the joint capsule, ligament and tendon sheath
.
The cyst contains colorless transparent or orange, yellowish thick mucus, the cyst wall is regular, for dense and tough fibrous connective tissue, cyst is unilocular as more common, patients are mostly young adults, women are more common
.
Onset is slow, with a round lump visible at the site of onset, with a slight soreness
.
The patient has a localized mass and mild soreness, but the cyst wall is disorganized, which can temporarily rule out the diagnosis
.
2.
Special rheumatoid arthritis: AS can be combined with rheumatoid arthritis, when the patient's elbow joint is involved, it is necessary to be vigilant about whether it is combined with rheumatoid arthritis, but the patient has no small joints of both hands, rheumatoid factor, anti-CCP antibody are negative, imaging has not seen positive changes in the joint surface, joint fusion and joint space narrowing, so the diagnosis can also be ruled out
.
3.
Synovial chondroma: Synovial chondroma is also known as synovial chondrocartilage metaplasia, is a rare benign joint disease, characterized by the formation of cartilage nodules on the synovium, the cartilage body of synovial chondroma is mostly sandy, can form intra-articular free bodies (also known as articular mice), palpable on the body surface, imaging performance is very typical, basically seen will not forget
.
The patient's presentation is inconsistent and the diagnosis
is excluded.
4.
Hemophilia: Hemophilic arthritis can also lead to joint swelling and pain, which is joint degeneration caused by repeated bleeding in the joints of hemophilia patients.
It is more common in knee, elbow, ankle, shoulder and other joints, and is more common in children
aged 8~10 years.
Patients often have bleeding from other parts such as the nasal cavity, abnormal coagulation function, and bloody effusion
can be seen on joint puncture.
The diagnosis is also excluded
in combination with the patient's age and the absence of bleeding from other sites.
5.
Infectious arthritis: infectious arthritis refers to the joint cavity caused by bacteria, viruses and other microorganisms invading the joint cavity caused by joint inflammation, which will cause local swelling and pain in the joint, but the swelling and pain are very obvious, and accompanied by systemic changes such as systemic fever, hemoptysis, night sweats, weight loss, etc.
, and PCT is normal, the diagnosis can be ruled out
.
6.
Synovial sarcoma: Synovial sarcoma is a malignant tumor
originating from the soft tissues of joints, synovial membranes and tendon sheath synovium.
Large joints of the limbs are the preferred site, and can also occur on
the muscular membrane and fascia of the forearm, thigh, and lower back.
Multiple local masses protruding significantly from the skin, pain, and limited mobility may occur, with multiple masses on imaging and low-density signals
.
The mass is slightly protruding, slightly sore, and radiographic findings are inconsistent
.
7.
Solid tumor bone metastasis: the patient has no mass changes, and the tumor markers are normal, so the diagnosis
can also be ruled out.
8.
Lymphoma: Lymphoma will also have joint swelling and pain, but the patient has no abnormal enlarged lymph nodes in the examination, and there are no systemic changes such as fever, so lymphoma can also be excluded
.
The "gold standard" – biopsy, ultimately to help clarify the diagnosis
The above diagnoses have been ruled out, it seems that it still depends on biopsy! Finally, the patient decided to undergo an ultrasound-guided puncture biopsy of the left elbow joint, and the results are shown in the figure: Figure 4: Pathological biopsy results, the red arrow points to hemosiderin deposition, and the green arrow points to foamy histiocyte infiltration
turned out to be AS villinododular synovitis (PVNS) with pigmentation!
What exactly is PVNS?
PVNS refers to unexplained chronic synovial nodular or villous hyperplastic lesions
in joints, tendon sheaths, and bursa.
It was first proposed by Jaffe et al.
in 1941, so it is also called Jaffe syndrome
.
Features: synovial hyperplasia of joints, tendon sheaths and bursa, accompanied by pigmentation (hemosiderin) deposition, often repeated bleeding and swelling of the joints, sometimes invasive into adjacent bone tissue, affecting joint function
.
Etiology: related to
inflammation, trauma, tumors, abnormal expression of genes and proteins.
▌Clinical manifestations
- The most common clinical presentation is a soft-tissue mass and pain
. - Common clinical symptoms of intra-articular PVNS are pain and diffuse swelling, sometimes joint strangulation
. - Prone parts: PVNS in the joint is easy to invade large joints, mainly knee joints, followed by hip joints, shoulder, elbow, ankle joints can also occur, other such as knuckles, talus joints, temporomandibular joints, vertebral facet joints have also been reported
.
PVNS has two manifestations: diffuse and limited
Diffuse: total synovial involvement
of 1 interarticular compartment or intraarticular.
Diffuse type has a high rate of recurrence after surgery, which can lead to chronic diseases and affect the normal function
of the joints.
Limited: local synovial involvement
.
Localized forms rarely recur after surgical resection
of marginal tissue.
: most of the joint fluid is bloody, but it can also be orange
.
2.
X-ray: nodular PVNS of the hand can be manifested as soft tissue swelling and bone invasion
.
The bone destruction area has well-demarcated sclerotic margins, and this bone destruction is the result of direct spread of the lesion pressing on adjacent bone, and does not indicate malignancy
.
3.
CT: CT scan can show the extent of hemosiderin, synovial lesions, and cystic changes and invasion of
bones.
4.
MRI: The characteristic T 1 W1 andT2W1 on MRI are all low signals, which are caused by
the paramagnetic effect of hemosiderin.
This sign is a reliable diagnostic basis
for PVNS.
▌PVNS diagnostic criteria
PVNS clinical manifestations and imaging examination are not specific; Definitive diagnosis relies on synovial histopathology
.
Blue is hemosiderin deposition and green is foamy histiocyte
.
Figure 5: Synovial pathological examination
- Surgical treatment
- Postoperative adjuvant therapy
- Cryotherapy
- Biologically targeted therapy
.
For PVNS, the consultation of the Department of Orthopedic Oncology indicated that the tumor lesions were diffuse, widespread, difficult to operate, and had a high risk of recurrence, and symptomatic drug treatment was given, follow-up observation, and joint replacement was performed if the disease progressed further
.
Dr.
Wang Lifang believes that if patients with AS have peripheral joint involvement, they need to be vigilant about PVNS, and can improve CT, magnetic resonance, and puncture biopsy if necessary to confirm the diagnosis
.
Patients with PVNS have low back pain and should be noted for the presence or absence of AS, with further exceptions
that can complete CT of the sacroiliac joint.
Finally, Dr.
Wang Lifang emphasized, "In short, do not miss any clues, provide patients with early and correct treatment, and avoid irreparable joint damage!" ”
Expert reviews
The patient's AS diagnosis is clear, when peripheral joint accumulation occurs in time, according to monist thinking, it is easy to think that AS affects peripheral joints, but Dr.
Wang Lifang did not preconceive, but improved CT and MRI in time for the swelling and pain of the soft tissues of the left elbow joint, and found imaging manifestations different from AS peripheral joint involvement.
Further stripping away the cocoons and finally clarifying the diagnosis is worth learning
from the rigorous and responsible attitude towards patients.
In rheumatology, for patients with monoarthritis, especially chronic synovitis of the knee joint, it is often necessary to identify it, PVNS is most common in the knee joint, elbow, hip, ankle and other parts can also appear, but relatively rare, clinically there are no characteristic manifestations, MRI is suggestive, and synovial biopsy pathology is the gold standard
for diagnosis 。 Clinically, PVNS is very easy to be misdiagnosed as spondyloarthritis and seronegative rheumatoid arthritis and given antirheumatic drugs, and the treatment effect is not ideal, which may eventually lead to irreversible joint destruction
in patients.
Therefore, for chronic arthritis with single joint involvement, rigorous differential diagnosis is more needed, especially if the case is AS combined with PVNS, which is clinically rare, more difficult to identify, and very easy to omission, so that patients can not be diagnosed and treated
in time.
This case has opened up clinical ideas, suggesting that we cannot think inertia clinically, do not let go of every suspicious symptom and sign, carefully screen them, make a clear diagnosis in time and give effective treatment plans to protect the patient's joints
.
Interpreting physicians
Wang Lifang, attending physician
Attending physician, Department of Rheumatology and Immunology, Peking University Shougang Hospital
Master of Medicine
In 2016 and 2019, he won the third prize of Guangxi Science and Technology Progress Award
Youth member of the 3rd Rheumatology Professional Committee of Beijing Society of Integrative Medicine
Commenting physician Li Ru Chief physician
Chief Physician, Department of Rheumatology and Immunology, Peking University People's Hospital, Associate Professor
Member of the Standing Committee and Deputy Secretary-General of the Rheumatology and Immunology Committee of the Chinese Association of Women Physicians
Executive Editor of Rheumatology & Autoimmunity
Member of the Standing Committee of the Rheumatology and Immunology Committee of the Rheumatology and Immunology Branch of the Chinese Medical Doctor Association
Member of the editorial board of the Special Issue of Medical Reference Journal Rheumatology and Immunology
This article is compiled from the "Medical Doctor Station" online video course, included in the "Rheumatology Clinical Classic Case Collection", for the full version of the course content, please log in to the Doctor Station APP to watch
.
Webusers who have downloaded the "Medical Doctor Station" can directly scan the QR code below to view the course
Source of this articleMedical Rheumatology and Immunology Channel Author of this articleFuji This article reviewChen Xinpeng Deputy Chief Physician Responsible EditorOrange
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