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Despite the diagnosis of rigidity, rheumatologists should not ignore these two conditions!

 Last Update: 2022-11-05
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Synopsis:
A 29-year-old woman presents with "lower limb discomfort with low back pain for 10 years and polyarticular pain for 3 months
".

The patient went to the hospital for examination 10 years ago when he developed lower limb discomfort and low back pain, which showed positive HLA-B27, other results are unknown, doctors diagnosed ankylosing spondylitis (AS), and gave sulfasalazine and meloxicam oral treatment
.
After the medication, the patient's joint pain was reduced, and the drug was stopped on his own after about 2 years, and there was no obvious recurrence
during the period.
Obviously, this visit is a "recurrence of old diseases", what can we learn from such a well-diagnosed AS patient? Let's follow the wonderful explanations of Attending Physician Zhang Yang (Interpreting Physician) of Aerospace Center Hospital and Chief Physician Liu Xu (Commenting Physician) of Peking University People's Hospital, and continue to read on!

Case introduction

The patient had joint pain again before 1 month, involving the waist and hips, back back, neck, shoulder joints, left palm root, upper sternum, left hip joint, and knee joints.

No fever, rash, no dry mouth, dry eyes, no ophthalmia, pain can be reduced
after self-administration of celecoxib treatment.

Good fellow, this painful part can be said to be all over the body, seriously affecting the normal life of
the patient.

Let's take a look at the patient's past history: diagnosed with iron deficiency anemia for half a year, had red blood cell and iron transfusions, and is currently taking polysaccharide iron complex capsule treatment
.
Regarding iron deficiency anemia, the doctor learned that the female patient hardly ate meat and eggs, so the consideration may be related to
diet.

Personal history: no smoking and alcohol addiction, regular menstruation, married, 1 child in good health, healthy parents
.

▌Physical examinationBody
temperature: 36.
1 °C, pulse: 84 times / min, breathing: 20 times / min, blood pressure: 104/72mmHg, clear mind, spirit.

No anemia, no paleness of the eyelid conjunctiva, rosy lips, no rash, no abnormalities in the heart, lungs and abdomen
.

Positive lumbosacral and upper sternal tenderness.

The distance between the ground and the occipital wall is 0cm, the Schober sign is negative, and the character 4 sign is positive
.

▌Auxiliary inspection

Blood routine: white blood cells 7.
20×10⁹/L; Hemoglobin: 113g/L; hematocrit: 0.
374, mean red blood cell volume: 75.
9fl, mean hemoglobin content: 22.
9pg, mean hemoglobin concentration: 301.
0 g/L; Platelets: 314×10⁹/L;
Biochemistry: potassium: 3.
93mmol/L; Sodium: 141mmol/L; Urea: 3.
5mmol/L; Blood creatinine: 39.
3μmol/L; Uric acid: 287.
8μmol/L; alanine aminotransferase: 18.
6 IU/L; aspartate aminotransferase: 18.
0 IU/L; Total protein: 72.
10 g/L; albumin: 37.
50 g/L;
Routine occult blood test in stool: weakly positive;
no abnormalities in urine routine;
ESR: 60 mm/h; CRP：72.
96 mg/L； Ferritin: 194.
63 ng/ml;
Immunoglobulin G: 1444.
00 mg/dl; Immunoglobulin A: 486.
00mg/dl; Complement C3: 143.
00mg/dl;
HLA-B27 positive;
There were no abnormalities in coagulation function, tumor markers, thyroid function, glycated hemoglobin, and TB-spot;
There were no abnormalities
in the ANCA combination, lymphocyte subsets, antinuclear antibody profiles, and antiphospholipid antibodies.

Fig.
1 Positive lateral bitmap
of the patient's X-rayFig.
2 CT of the patient's sacroiliac joint

CT chest: old lesions of both lungs, splenomegaly;
Abdominal ultrasound: no obvious abnormalities in the accessory spleen, liver, gallbladder, pancreas, and spleen;
Urinary ultrasound: no obvious abnormalities in both kidneys and bladder;
Cardiac ultrasound: at rest, there are no obvious abnormalities in cardiac structure and blood flow;
Sacroiliac joint CT: consistent with AS changes, recommended in combination with clinical;
X-ray of the lumbar spine: no abnormalities;
Hip magnetic resonance imaging (MRI): consider ischemic necrosis of the left femoral head and joint capsule effusion
.

▌Clinical diagnosis
1.
AS;
2.
Iron deficiency anemia;
3.
Aseptic necrosis
of the left femoral head.

With such a clear diagnosis, what else can we learn?

Indeed, as seen above, the patient's diagnosis is very clear: young woman, 10 years of medical history, recent exacerbation, clinical manifestations of polyarticular pain, involving the central axis and peripheral joints, characterized by inflammatory low back pain, positive HLA-B27, sacroiliac joint x-ray, CT showing sacroiliitis changes, according to the New York standards revised in 1984, can be diagnosed as AS
.

Figure 3 New York standards
in 1966 and 1984 But in addition to the diagnosis of AS, let's also look at the association
of the patient's other two diagnoses with AS.

▌Attending physician Zhang Yang of iron deficiency anemia introduced that the patient has eating habits that do not like to eat meat and eggs, which may be related to anemia
.

In the following review session, Chief Physician Liu Xu added
to this.
Patients with inflammatory arthropathy may have iron deficiency due to inadequate iron
intake or insensible gastrointestinal losses for various reasons.
In addition, long-term inflammation can also lead to a decrease in the sensitivity of red blood cells to erythropoietin, which can lead to anemia
of chronic diseases.
This type of anaemia can recover
after the patient's condition is under control.

▌The diagnosis of aseptic necrosis
of the left femoral head is based
on the patient's left hip pain and hip MRI results.
However, attending physician Zhang Yang also emphasized that in fact, AS can also involve the hip joint, so it may be indistinguishable
from aseptic necrosis of the femoral head.
The patient does not have common risk factors for aseptic necrosis of the femoral head (long-term hormonal drug history, alcohol use, trauma, etc.
).

Therefore, strictly speaking, hip lesions are also related to
AS.

Chief Physician Liu Xu also shared her experience
.
Many patients with AS develop hip joint lesions, which is an important cause
of abnormal function in AS patients.
This lesion is indistinguishable from aseptic necrosis of the femoral head in both imaging and clinical manifestations, and does not make too many clinical distinctions
.
But we can appreciate the difference
between the two in some subtle ways.
If the hip joint is caused by AS, it is often a lesion on the joint surface, such as bone destruction or bone erosion, and this change is more concentrated on the articular surface and acetabular surface
.
Aseptic necrosis of the femoral head may first manifest on MRI, the "crescent sign", which is a lesion
of the entire femoral head caused by ischemia.
It may start from a more central site, and it has more typical manifestations on MRI in the early stage, but it is difficult to distinguish
it from AS lesions in the later stage due to the collapse of morphology.

What else should be paid attention to in the treatment of AS?

AS is a chronic inflammatory disease that mainly affects the axial joints and can affect peripheral joints, which belongs to the category
of spondyloarthritis (SpA).
The prevalence of AS in China is 0.
3%, mostly occurring at 10~40 years old, male: female = (2~3): 1
.
Pathogenesis is related
to genetic factors (HLA-B27), environmental factors (intestinal infection), and cytokines.
Pathologic manifestations are sacroiliitis (specific findings), enthesitis, peripheral synovitis
.
In general, AS can involve the following parts: Fig.
4 Sites
that may be involved in AS ■ Central axis involvement:

Inflammatory back pain: one of the hallmark features of AS, if the following 4 items are met, it can be judged: (1) the age of onset is less than 40 years old; (2) occult onset; (3) improvement after exercise; (4) It cannot be improved after rest; (5) Night pain (improves after waking up).

However, it should be noted that not all patients with inflammatory low back pain can be diagnosed with AS;
Sacroiliitis;
alternating hip pain (mechanical low back pain can also be caused);
Inflammation of the anterior chest;
morning stiffness (early atypical);
Spinal rigidity
.

■Peripheral joint involvement:

25%~45% of AS patients first found peripheral joint involvement;
The younger the age of onset, the more pronounced peripheral joint involvement and the higher the disability;
There are more joints of the lower limbs than joints of the upper extremities;
Single/oligoarticular involvement is more common than polyarticular;
There is more asymmetry than symmetry;
Intermittent onset;
Joint bone destruction is uncommon
.

■Enthesitis:

The peripheral area often involves the plantar fascia and the insertion point of the Achilles tendon on the calcaneus, and can also be seen in the tibial tuberosity, ischial tuberosity, and costal cartilage junction;
In the spine, it can be seen in the attachment of bursa and ligaments, intervertebral discs, costopone joints, costover-transverse process joints, parapyramidal ligaments, and bone attachments of intervertebral disc
ligaments.

■Extra-articular manifestations:

Uveitis;
Involvement of the heart (involving heart valves, conduction system, decreased cardiac function, etc.
), lungs, kidneys, etc
.

Regarding the management of AS, the guidelines of the International Association for Spondyloarthritis (ASAS) and the European League against Rheumatism (EULAR) on axial SpA updated this year put forward 5 general principles and 15 specific recommendations
.

Table 1: The specific 15 recommendations of ASAS and EULAR on the 5 general principles of axial SpA revolve around the following three areas:
■ Non-pharmacological treatment

patient education;
lifestyle modifications;
Sports, physiotherapy
.

■Drug treatment

Non-steroidal anti-inflammatory drugs (first-line drugs): generally choose a non-steroidal anti-inflammatory drug, observe the efficacy of 2~4 weeks, if it is not good, switch to another, generally not recommended to use two drugs at the same time, the risk of the patient's medication needs to be evaluated before medication;
Antirheumatic drugs (DMARDs): 1.
Traditional DMARDs; 2.
Biological DMARDs (TNF-α inhibitors, IL-17 inhibitors); 3.
Targeted synthesis of DMARDs (JAK inhibitors);
Glucocorticoids
.

■Surgical treatment

Hip replacement, spinal correction surgery
.

In this case introduced by attending physician Zhang Yang, the patient used the non-steroidal anti-inflammatory drug celecoxib before admission, and still used celecoxib 0.
2g bid po at the beginning of admission, but his routine occult blood test was weakly positive, so it was gradually stopped after the initial control of the condition
.
And start sulfasalazine, gradually increase the dose to 1g bid po, and at the same time, perform subcutaneous injection
of recombinant human tumor necrosis factor type II receptor-antibody fusion protein twice a week.
Considering that the patient had anemia, 0.
2g qd po of
ferrous succinate extended-release tablets was given.

This is a very typical case in terms of symptoms, imaging, and auxiliary examinations, which can help everyone visualize the onset and treatment process
of AS patients.
Chief Physician Liu Xu reminded: When inflammatory back pain occurs, accompanied by peripheral joint swelling, it is necessary to go to the rheumatology department in time for examination to achieve early diagnosis and treatment
.
The majority of doctors and friends should also pay attention to the fact that even if the MRI performance of the patient is typical, it should be fully combined with clinical and laboratory manifestations for diagnosis
.
Finally, I hope that the majority of doctor readers can gain something from it! Reviews expert profiles

Liu Xu Chief Physician

Chief Physician, Department of Rheumatology and Immunology, Peking University People's Hospital, Associate Professor, Master Tutor
Doctor of Medicine, Peking University Health Science Center
Master of Science, Department of Biology, Hong Kong University of Science and Technology
Member of the Department of Rheumatology and Immunology, Peking University
Member of the Infectious Disease Group of the Rheumatology and Immunology Committee of the Cross-Strait Medical and Health Exchange Association
Member of the Rheumatology Committee of Beijing Society of Integrative Medicine

Interpret expert profiles

Zhang Yang Attending physician

Master of Department of Rheumatology and Immunology, Aerospace Center Hospital
Familiar with the diagnosis and treatment of common diseases in rheumatology and immunology such as systemic lupus erythematosus, rheumatoid arthritis, ankylosing spondylitis, etc

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