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Chapter 6 · Rheumatology

Tada Y, etal. Flexor tenosynovitis of the ahnd as an initial

manifestation of systemic lupus erythematosus. Mod Rheumatol. 2000;10:173–5.

Tehranzadeh J, etal. Advanced imaging of early rheumatoid

arthritis. Radiol Clin North Am. 2004;42:89–107.

Unlu Z, etal. Magnetic resonance imaging  ndings in a case

of remitting seronegative symmetrical synovitis with pitting edema. Clin Rheumatol. 2005;24:648–51.

Weissman BNW, etal. Prognostic features of atlantoaxial sub-

laxation in rheumatoid arthritis patients. Radiology. 1982;144:745–51.

Wu Y, etal. Jaccoud’s arthropathy and psoriatic arthritis, a rare

association. Rheumatol Int. doi:10.1007/s00296-009- 1017-1.

Zolcinski M, etal. Central nervous system involvement as a

major manifestation of rheumatoid arthritis. Rheumatol Int. 2008;28:281–3.

6.2 Ankylosing Spondylitis

(Marie–Strümpell Disease)

Ankylosing spondylitis (AS) is a chronic, progressive in ammatory disease of unknown origin that a ects the axial skeleton (vertebral column plus the pelvis) and is characterized by bilateral sacroiliitis, sti ness of the axial joints (ankylosis), and syndesmophytes formation.

AS is a rheumatoid factor seronegative arthritis that is positively associated with HLA-B27. HLA stands for human leukocyte antigen (HLA) system. In the body, there are two classes of HLA antigens. Class I HLA is expressed by the human cells for histocompatibility, so the body knows that

Signs on Radiograph

5 Sacroiliitis involvement is almost always in a

bilateral and symmetrical fashion in AS (90 % of cases). However, unilateral involvement may occur in 10 % of cases. Radiological findings include multiple small erosions (rat bite erosions) along the iliac side of the joint. In the advanced stage of the disease, the erosions increase in size and widen the sacroiliac joint. Later, sclerosis of the sacroiliac joint occurs (

5 Vertebral bodies squaring is an early manifestation of

AS that arises due to inﬂ ammation of the peripheral ﬁ bers of the annulus ﬁ brosis at their attachment to the upper and lower corners of the vertebral bodies (enthesitis). Enthesitis means inﬂ ammation of the entheses, the location where a tendon or a ligament is inserted into a bone. Erosions of the vertebral body at these areas make the vertebral body look square in shape (

5 Syndesmophytes are paravertebral ossifications

that resemble osteophytes, except that they arise vertically from one vertebra to the other (

. Fig. 6.2.3 ), while osteophytes run in a horizontal

fashion along the vertebral bodies. When the syndesmophytes are diffusely affecting the vertebral column, the vertebral column is said to have a “bamboo spines appearance” ( Syndesmophytes are ossifications of the annulus fibrosus–longitudinal ligament complex as a healing process after enthesitis.

. Fig. 6.2.1 ).

. Fig. 6.2.2 ).

. Fig. 6.2.4 ).

these cells are its own cells. Class I HLA antigens have an important role in transplant rejection. Class II HLA antigens are expressed by the immunocompetent cells including macrophages, Langerhans cells, B cells, and some T cells. HLA- B27 antigen is associated with AS in 90 % of cases. However, only 5 % of patients with positive HLA-B27 develop AS.

Patients with AS o en present with back sti ness, low back ache, and discomfort in the thighs and buttocks. Extraskeletal manifestations include anterior uveitis, ascending aortitis, and bronchiolitis obliterans with organizing pneumonia (cryptogenic organizing pneumonia).

Cauda equina syndrome is an uncommon complication of AS. Patients classically present with symptoms related to compression of the cauda equina such as low back and lower extremities pain, impotence, over ow incontinence, cutaneous sensory defects (paresthesia), and motor dysfunction. Up to 30 % of patients describe severe burning or shooting pain in the lower limbs.

. Fig. 6.2.1 Anteroposterior plain radiograph of the hip of a

patient with advanced ankylosing spondylitis (AS) shows complete sclerosis of the sacroiliac joints bilaterally ( arrowheads )

6.2 · Ankylosing Spondylitis (Marie–Strümpell Disease)

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. Fig. 6.2.2 Lateral plain radiograph of the thoracic vertebrae

in advanced stage of AS shows squaring of the vertebrae

. Fig. 6.2.4 Anteroposterior plain radiograph of the thoracic

vertebrae shows the classic appearance of bamboo spines

5 Dagger sign is longitudinal radio-opaque line seen

along the vertebral column representing calcification of the supraspinous ligament (

. Fig. 6.2.5 ).

5 Trolley track signs are three dense radio-opaque

lines seen along the vertebral column representing calcification of the supraspinous and ankylosis of the facets joints.

. Fig. 6.2.3 Anteroposterior plain radiograph of the thoracic

vertebrae shows syndesmophytes ( arrowheads )

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Chapter 6 · Rheumatology

. Fig. 6.2.5 Anteroposterior plain radiograph of the thoracic

vertebrae shows the dagger sign ( arrowheads )

Signs on MRI

5 Cauda equina syndrome typically presents as

enlargement of the thecal sac, multiple dorsal diverticula, with asymmetric scalloped erosions of the bony canal.

5 Romanus lesion is enthesitis at the insertion of the

annulus ﬁ brosus–longitudinal ligament complex. There is low T1 signal intensity, high T2 signal intensity, and marked contrast enhancement within the annulus ﬁ brosus at the discovertebral junction, indicating active enthesitis ( active enthesitis starts to heal, it forms syndesmophytes.

5 Anderson lesion is a focal erosive change in the

vertebral endplate that resembles bacterial discitis (

. Fig. 6.2.7 ). Typical features of Anderson lesion

include disk space narrowing, focal bone destruction at the vertebral endplate adjacent to the disk, surrounding sclerosis, and local kyphosis. Diﬀ erentiation between bacterial discitis and Anderson disease can be diﬃ cult in patients with AS. However, the vertebral disk is typically involved in bacterial discitis, while in Anderson lesion, the disk signal is generally preserved or shows degeneration. Moreover, perivertebral eﬀ usion and intradiscal eﬀ usion are commonly found with bacterial discitis, whereas they are rare with Anderson lesion. After

. Fig. 6.2.6 ). When the

two adjacent vertebrae (Romanus lesion) ( arrowheads )

. Fig. 6.2.6 Sagittal thoracic T1W ( a ) and STIR ( b ) MRI show enthesitis at the anterior superior and anterior inferior vertebral endplates of

6.2 · Ankylosing Spondylitis (Marie–Strümpell Disease)

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contrast injection, both Anderson lesion and bacterial

discitis show contrast enhancement. Bacterial discitis

high T2 signal intensity is due to hyperemia and

edema, while enhancement in Anderson lesion is due

to granulation tissue formation.

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. Fig. 6.2.7 Sagittal lumbar T1W MR illustration demonstrates

Anderson’s lesions ( arrowheads )

D i ﬀ erential Diagnoses and Related Diseases

– SAPHO syndrome is a rare musculoskeletal disease of

unknown origin characterized by S ynovitis, A cne, P ustu- losis of the palmar and plantar skin surfaces, H yperostosis of the bones, and O steitis. Patients with the adult form of SAPHO syndrome usually present with unilateral sacroiliitis and syndesmophytosis that may mimic the radiographic picture of AS.

– Di use idiopathic skeletal hyperostosis ( DISH ) is a disease

characterized by multisegmental vertebral fusion due to ligamentous calci cation and ossi cation.  e disease is commonly seen in the cervical and the thoracic vertebrae. Patients are usually above 70 years of age presenting with neck pain and sti ness. DISH can be mistaken with AS. Characteristic radiological signs of DISH include  owing vertebral ossi cation of at least four contiguous vertebral bodies, broad band of ossi cation along the anterolateral aspect of each vertebra (. Fig. 6.2.8 ), absence of degenerative disk disease, and absence of sacroiliac joints disease.

. Fig. 6.2.8 Lateral plain radiograph of the cervical vertebrae shows

multisegmental vertebral fusion due to ligamentous calciﬁ cation and ossiﬁ cation due to diﬀ use idiopathic skeletal hyperostosis (DISH) ( arrowheads )

Further Reading

Agarwal K, etal. Fine needle aspiration cytology of gouty

tophi with review of the literature. J Cytol. 2007;24:142–5.

Cabot J, etal. Tophaceous gout in the cervical spine. Skeletal

Radiol. 2005;34:803–6.

Chang PC, etal. Tophaceous gout of the  rst costochondral

junction in a heart transplant patient. Skeletal Radiol. 2006;35:684–6.

. Fig. 6.3.4 Coronal T1W ( a ), STIR ( b ), and T1W postcontrast foot MRI of a patient investigated for gout with foot pain localized to the

big toe show hypointense signal intensity due to edema of the ﬁ rst metatarsal head in ( a ), hyperintense T2 signal intensity in ( b ), and marked contrast enhancement ( c ) of the ﬁ rst metatarsal head and the surrounding soft tissues due to active inﬂ ammatory process ( arrowheads )

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Chapter 6 · Rheumatology

Calcium Pyrophosphate Dihydrate Crystal Deposition Disease

CPPD, also known as pseudo - gout and chondrocalcinosis , is a disease characterized by calcium pyrophosphate crystal deposition within the articulating cartilage , leading to carti- lage in ammation and later to joint destruction in a similar fashion to gout arthritis.

CPPD is classi ed based on its etiology into hereditary, idiopathic, or secondary to metabolic disorders (e.g., vitamin D intoxication).  e disease is age related, with an incidence of 5 % in patients >70 years and nearly 50 % in patients

. Fig. 6.3.5 Axial T1W brain MR illustration demonstrates bilateral

caudate nucleus atrophy ( arrowheads ) in a patient with Lesch–Nyhan syndrome (LNS)

>90years. Many patients present with gout-like arthritic episodes characterized by joint synovitis, malaise, and fever that last from 1 day to 4 weeks. Up to 50 % of patients develop progressive degeneration of multiple joints.  e most frequently involved joints are the knees, wrists, metacarpophalangeal joints, and the hips.

Pyrophosphate deposition involves both hyaline cartilage and  brocartilage joints like symphysis pubis, annulus of the spine, triangular  brocartilagenous complex (TFCC) of the wrist, and menisci. CPPD can occur in high incidence with other diseases like gout, hyperparathyroidism, and hemochromatosis.

CPPD diagnosis is established by identifying the pyrophosphate crystals within the synovial  uid a er aspiration. Plasma and uric acid levels of pyrophosphate are typically not elevated (di erential point from gout).

Harris JC, etal. Craniocerebral magnetic resonance imaging

measurments and  ndings in Lesch-Nyhan syndrome. Arch Neurol. 1998;55:547–53.

Jacobson JA, et al. Radiographic evaluation of arthritis:

in ammatory conditions. Radiology. 2008a;248:378–89.

J a j i ć I, etal. Gout in the spine and sacro-iliac joints: radio-

logical manifestations. Skeletal Radiol. 1982;8:209–12.

Monu JUV, etal. Gout: a clinical and radiological review.

Radiol Clin North Am. 2004;42:169–84.

Rosenfeld DL, et al. Serial renal songraphic evaluation in

patient with Lesch-Nyhan syndrome. Pediatr Radiol. 1994;24:509–12.

6.4 CPPD and HADD

Calcium pyrophosphate dihydrate crystal deposition disease (CPPD) and hydroxyapatite crystal deposition disease (HADD) are diseases characterized by deposition of insoluble crystals within the joints and periarticular so tissues, initiating in ammatory destructive reaction. Other clinically important calcium-containing crystal deposition diseases include tricalcium phosphate (TCP) and octacalcium phosphate (OCP) diseases.

Signs on Plain Radiograph

5 Chondrocalcinosis : cartilage calcification is the

hallmark of CPPD. Chondrocalcinosis is usually observed in medial and lateral compartments of the knee, wrist TFCC, and the symphysis pubis (

. Fig. 6.4.1 ).

5 Pseudo - Charcot ’ s joint : severe joint destruction that

mimics Charcot’s joint may be observed occasionally.

5 Normal bone density with occasional subchondral

cysts.

5 SLAC wrist deformity : S capholunate A dvanced

C ollapse is a pathological situation characterized by loss of the cartilage between the scaphoid bone and the radius, causing the scaphoid to indent the radius and the capitate to collapse, thus disturbing the scapholunate joint articulation (

. Fig. 6.4.2 ).

5 Generalized chondrocalcinosis : a pathological

condition characterized by involvement of more than one group of joints with cartilage calcification (e.g., knees, wrists, plus vertebral disks).

6.4 · CPPD and HADD

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. Fig. 6.4.1 Anteroposterior knee radiograph shows

calciﬁ cation of the lateral meniscus due to CPPD chondrocalcinosis ( arrowhead )

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Hydroxyapatite Crystal Deposition Disease

HADD, also known as calci c periarthritis and peritendinitis calcarea , is characterized by hydroxyapatite crystal deposi-

tion in the so tissues, especially the tendons.

 e most characteristic feature of this disease is tendon

calci cation within the body, especially around the shoulder. Moreover, crystal deposition and calci cation tend to occur characteristically around the joints (periarticular). HADD can be sporadic, or associated with long-term hemodialysis for renal insu ciency.

Patients with HADD can be asymptomatic or present with recurrent attacks of arthritis in the area of crystal deposition. Shoulder pain is the commonest complaint since supraspinatus tendon calci cation is common in HADD.

HADD is characterized by three pathological phases: silent, mechanical, and adhesive.  e silent phase is characterized by crystal deposition that is completely within the tendon.  e mechanical phase is characterized by enlargement of the deposits with starting of impingement-like symptoms (e.g., bursitis).  e adhesive phase is characterized by generalized disability and limitation of motion. When the adhesive phase occurs in the shoulder, the condition is called adhesive capsulitis or frozen shoulder . Hydroxyapatite crystals are com- monly deposited in damaged tissues (dystrophic calci cation).

HADD calci cation is o en monoarticular, although it can be polyarticular. Involvement of the joints of the feet and toes is rare (<1 %).  ere are two syndromes associated with HADD due to crystal deposition around the joints: calci c periarthritis with bone resorption (acute HADD arthritis) and rapid destructive arthritis of the shoulder (Milwaukee shoulder syndrome).

Calci c periarthritis with bone resorption is characterized by in ammation of the calci ed focus with resorption of the bone beneath it.  e condition mimics bone sarcoma, especially if periostitis develops. Biopsy can be avoided if the location of the osteolytic lesion is characteristic of HADD (near a tendon insertion), and other manifestations of HADD exist in the body.

Milwaukee shoulder syndrome is a disease characterized by destructive shoulder arthropathy, bloodstained joint e usion (80 %), and chronic tears of the rotator cu tendon. Patients are typically elderly women with a mean age of 72 years. Symptoms range from none to severe shoulder pain with joint e usion. Most patients have symptoms dating from several years back. Bilateral shoulder involvement is common, and knees arthropathy is found in 50 % of patients.

. Fig. 6.4.2 Plain hand radiograph shows scaphoid indenting

the distal radius with sclerosis ( arrowhead ) and collapse of the capitate from its normal position ( arrow ) (SLAC wrist deformity)

D i ﬀ erential Diagnoses and Related Diseases

Crowned dens syndrome ( CDS ) is a rare clinical condition characterized by deposition of pyrophosphate or calcium hydroxyapatite crystals around the odontoid process of the

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Chapter 6 · Rheumatology

axis vertebra and its ligaments, especially ligamentum  avum. In ammatory signs and high erythrocyte sedimentation rate (ESR) are present in up to 30 % of cases.

 e patients o en present with acute attack of neck pain, neck rigidity, and fever, mimicking acute meningitis or spondylodiscitis. CDS a ects mostly females, with up to 45 % of cases found in patients above 85 years of age.

Signs on Radiographs

5 Calciﬁ cation of the supraspinatus and infraspinatus

tendons is a very characteristic feature of HADD (

. Figs. 6.4.3 and 6.4.4 ). The calciﬁ cation typically

starts in the site of tendon insertion or the critical zone. The critical zone is the part of the supraspinatus tendon 1 cm proximal to its insertion into the greater tubercle of the humerus.

5 Areas of calcifications are noticed in the

periarticular soft tissues.

5 Calcification within the carpal bones, ligaments,

and wrist tendons is commonly seen.

5 Always suspect HADD in a calcification that is

observed near a joint, at tendon insertion, near muscular attachment, or after trauma (dystrophic).

5 I n Milwaukee shoulder syndrome , there is glenohumeral

joint destruction, narrowing, and sclerosis. Upward subluxation of the humeral head can be seen, indicating long-standing rotator cuﬀ tendon disruption. Periarticular calciﬁ cation is noticed in 40 % of cases. Pseudoarthrosis between the humeral head, coracoid, and acromion is common. Knees involvement is similar to that of CPPD arthropathy.

5 I n crown dens syndrome , radio-opaque calciﬁ cations

with diﬀ erent sizes and shapes are seen around and above the superior part of the odontoid process, giving the shape of a “crown on a head” appearance. CDS can be mistaken with cervical block vertebra (Klipple–Feil anomaly type 1).