Chronic Gouty Tophi

After years of gouty attacks, patients will develop a chronic arthritis resulting in bone and cartilage destruction and deformity. Uric acid crystals deposit within and surrounding the joint causing a chronic destructive inflammatory process. X-rays characteristically show well demarcated "punched out" erosions.

Tophi at helix of ear.

 Tophi or deposits of uric acid crystals are often found around joints, in the olecranon bursa or at the pinna of the ear.

 




X-ray showing soft tissue swelling and erosion of 2nd PIP.

Systemic Lupus Erythematosus: A Companion to Rheumatology
Mosby; 1 edition (January 18, 2007) | ISBN: 0323044344 | 608 pages | PDF | 11 MB

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The very latest concepts in treatment

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Classification of inflammatory arthritis

Inflammatory arthritis is generally classified into seropositive and seronegative groups. These are based on the presence of rheumatoid factor, an immunoglobulin which reacts with gamma globulin, in the blood of the majority of patients with seropositive disease and in a small minority of patients with seronegative disease. The prototype seropositive form of arthritis is rheumatoid arthritis. Other members include the group of conditions labelled collagen vascular diseases, such as systemic lupus erythematosus, scleroderma, vasculitis, Sjogren’s syndrome. Only rheumatoid arthritis will be considered in this paper.

Among the seronegative inflammatory joint diseases is a group labelled spondyloarthritis. This condition is characterized by inflammatory disease of the joints of the back, both the sacroiliac joints and the apophyseal joints of the spine. Members of this group include ankylosing spondylitis, psoriatic arthritis, reactive arthritis, and arthritis of inflammatory bowel disease. While the prototype for this group is ankylosing spondylitis, psoriatic arthritis will also be discussed.

In addition to the presence of rheumatoid factor, there are extra-articular features which distinguish the seropositive from the seronegative forms of inflammatory arthritis.

A case of Inflammatory Arthritis

A 34 year old male has a long history of several subacute attacks of self-limited inflammatory arthritis. Crystals have been retrieved from a joint aspirate. Radiography of his knee is likely to demonstrate:

a) subperiosteal thickening
b) marginal articular erosions
c) central articular erosions
d) chondrocalcinosis
e) subchondral bony sclerosis

The correct answer is D

Explanation
Chondrocalcinosis is simply the term for the finding of radio-opaque crystals in hyaline or fibrocartilage. These salts may consist of calcium pyrophosphate dihydrate, dicalcium phosphate dihydrate or hydroxyapatite. Crystals may also be deposited in bursae, capsules, ligaments and tendons.
Chondrocalcinosis may accompany a variety of diseases, but is not a disease entity merely a physical sign thereof. Used properly, the term pseudogout refers to the clinical syndrome of one or more acute or subacute attacks of self-limited inflammatory arthritis caused by crystal-induced synovitis, proven by recovery of the crystals from a joint aspirate. Most people with chondrocalcinosis never have a single acute episode of pseudogout.

CPPD crystal deposition disease is the general term that encompasses chondrocalcinosis, pseudogout, a distinctive arthropathy and a host of variations. During acute attacks (pseudogout) of crystal-induced synovitis, the usual radiological findings are soft-tissue oedema and joint effusion. Chondrocalcinosis may or may not be present. The cartilage and articular bone are often normal. The chronic arthropathy is more distinctive. It is most frequent in the patellofemoral, radiocarpal metacarpophalangeal and elbow joints.

Although the findings superficially resemble osteoarthritis, there are several important differences beyond the unique distribution. The arthropathy is characterized by cartilage loss, multiple subchondral cystic rarefactions, structural collapse of the articular surface, and the presence of many small intra-articular fragments, all without much bone sclerosis. In addition to the usual target sites, these changes may be observed in the hips, knees and sacroiliac joints. Chondrocalcinosis, which does not have to accompany the arthropathy, is seen most commonly in the large joints such as the knee, hip and shoulder as well as the symphysis pubis.

Sclerodactyly in Systemic Scleroderma

Systemic scleroderma often affects the hands. The initial stage is swelling (edema), which can last for weeks, months, or years. Often the swelling is intermittent and worse in the morning. It can cause the fingers to look like sausages, with far fewer wrinkles. Skin tightness in the hands can make it impossible to pinch the skin on the fingers.
In some people, the fingers eventually begin to harden from fibrosis, and curl inward. They may then become frozen in this clawed position, which is referred to as "sclerodactyly."

So,When the skin on the fingers become tight, stretched, wax-like, and hardened it is called sclerodactyly. Sclerodactyly is commonly associated with atrophy of the underlying soft tissues.

Not everyone with scleroderma develops this degree of skin hardening. However, it is this symptom that has earned scleroderma the nickname of 'the disease that turns people to stone'.

Rheumatoid nodules appearance

These are a focus of central fibrinoid necrosis surrounded by a palisade of epitheloid cells, occurring as a typical feature of sero-positive rheumatoid arthritis..............

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Multiple Beau's Lines

A 69-year-old man was referred for the evaluation of changes to his fingernails that developed after the initiation of chemotherapy with docetaxel–cisplatin–fluorouracil for gastric cancer. Physical examination revealed multiple white lines on the fingernails, known as Beau's lines.
The lines corresponded to the start of each chemotherapy cycle, with the distance between the lines proportional to the interval between the cycles.
One white line showed less severe dystrophia than the others (arrow); on reviewing the medical records, it was noted that the corresponding chemotherapy cycle consisted of docetaxel–fluorouracil only, owing to the patient's compromised renal function at the time, whereas all the other cycles consisted of the complete docetaxel–cisplatin–fluorouracil regimen.

Cytotoxic chemotherapeutic agents can induce the temporary arrest of proliferative function of the nail matrix, which may be manifested as multiple Beau's lines in the nail plate. This nail pattern can provide insight into the interval between and intensity of chemotherapy cycles.

How to diagnose SLE by "MD SOAP 'N HAIR"

MALAR RASH

SLE (Systemic Lupus Erythematosus) diagnosis  !!

"MD SOAP 'N HAIR":   ...........

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Swan-neck deformity

A swan-neck deformity, defined as proximal interphalangeal (PIP) joint hyperextension with concurrent distal interphalangeal (DIP) joint flexion, occurs in approximately 50% of patients with rheumatoid arthritis RA. However, swan-neck deformity is not unique to RA, because it may also be congenital or traumatic in nature.

It may also caused by:
# mallet finger (due to rupture of lateral slips at their junction with bone)
# cerebral palsy (due to muscle imbalance)
# congenital joint laxity

The pathophysiology of the swan-neck deformity begins with flexor synovitis, which increases the flexor pull on the MP joint. Constant efforts to extend the finger against this pull lead to stretching of the collateral ligaments and the volar plate at the PIP joint.

In a normal finger, intrinsic muscles (interosseous and lumbrical) insert into the lateral bands and serve as flexors of the MP joint and extensors of the PIP and DIP joints by being located volar to the MP joint axis and dorsal to the PIP and DIP joint axes.

In a rheumatoid finger, the lateral bands are constrained in their dorsal position, upsetting the flexor-extensor balance. In this position, the lateral bands increase the pull of the long extensor tendon's central slip, which attaches to the dorsal base of the middle phalanx. The increase of flexor profundus tension resulting from hyperextension of the PIP joint leads to a reciprocal flexion of the DIP joint. Progressive disease causes joint destruction and fixed contracture.

Kienbock's Disease (Lunatomalacia)

.Avascular necrosis of lunate bone

· Predisposed in individuals engaged in manual labor with repeated or single episode of trauma

· Frequency
o Usually affects men aged 20-40 yrs.
o Mostly unilateral
o More often in right wrist

· Pathophysiology
o Vascular impairment due to acute or chronic injury
( Exact cause is unknown)
o Lunate develops osteonecrosis due to loss of blood supply, causing pain and stiffness in the wrist
o In late stages, the bone collapses eventually leading to degenerative changes and osteoarthritis in the radiocarpal joint

· Clinical findings
o Progressive pain
o Soft-tissue swelling of wrist


Imaging findings
o The disease can be staged based on radiographic findings

** Lichtman's Radiographic Classification of Kienbock's Disease
· Stage I - Normal radiograph
· Stage II - Sclerosis of lunate with possible decrease of lunate height on radial side only
· Stage IIIa - Lunate collapse, no scaphoid rotation
· Stage IIIb - Lunate collapse, fixed scaphoid rotation
· Stage IV - Degenerative changes around the lunate

§ The disease may also be associated with negative ulnar variance

Frontal view of the hand and wrist demonstrates sclerosis, irregularity
and collapse of the lunate (blue arrows) in Kienbock's Disease (Kienbock's Osteomalacia)

o Bone scan and MRI may be helpful early in the course of the disease when there are minimal radiographic findings.

Treatment:
** Initial therapy is conservative management
** Anti-inflammatory medications and splinting or casting
** Operative treatment is based on the stage of the disease and may involve
1-Revascularization procedures
2-Ulnar lengthening or radial shortening
3-Fusion or excision of carpal bones

Case of Wegener’s granulomatosis

A 43y old man presents with fever and arthritis. During the past 2 mo, he has been treated four times for a maxillary sinus infection. He also complains of the recent onset of hematuria. Which of the following is the most likely diagnosis?

• a.Churg-Strauss syndrome
• b.Wegener’s granulomatosis
• c.Lofgren syndrome
• d.Sjogren syndrome
• e.Sarcoidosis

** The answer is (b).

Wegener’s granulomatosis involves the upper airways (nasopharynx and sinuses) and the lungs, kidneys, and joints. The diagnosis is made by the clinical picture, a positive antineutrophil cytoplasmic antibody with a cytoplasmic staining pattern (C-ANCA), and biopsy showing necrotizing granulomas. The disease causes a systemic necrotizing arteritis and is fatal without treatment.
The typical history for Churg-Strauss syndrome(allergic angiitis and granulomatosis) is asthma followed by systemic vasculitis with eosinophilia (mnemonic is RAVE: Rhinitis, Asthma, Vasculitis, and Eosinophilia). Lofgren syndrome is a benign form of sarcoidosis that
causes bilateral hilar adenopathy, periarthritis of the ankles, and erythema nodosum of the anterior tibial regions of the lower extremities. Sjogren syndrome is a slowly progressive autoimmune disease that primarily affects middle-aged women; it affects the lacrimal and salivary glands,resulting in xerostomia and dry eyes. It may occur alone (primary) or in
association with other autoimmune diseases such as rheumatoid arthritis or systemic lupus erythematosus.

Oral Manifestations of Sjogren's Syndrome

Sjögren's (SHOW-grins) syndrome is an autoimmune disease that attacks the exocrine glands (i.e., moisture-producing glands) of the body, specifically the lacrimal and salivary glands. ..................

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ABC of Rheumatology

Musculoskeletal diseases are the most widespread chronic illness in the UK and one of the top 3 reasons why the over 45s visit their GP. The ABC of Rheumatology is a practical, introductory guide to the diagnosis, management and treatment of rheumatology and rheumatic diseases for the non-specialist. Providing detailed overviews of all major areas of rheumatology this fully revised fourth edition includes updated information on new treatments, therapies, evidence and guidelines.
An informative and practical source of knowledge, with highly illustrated chapters including boxed summaries, links to further information, reading and resources, this well established ABC title is an accessible reference for all primary care health professionals, GPs, junior doctors, medical students and nurses.



http://hotfile.com/dl/22918261/8354653/ABC_of_Rheumatology.pdf.html

A Clinician's Pearls and Myths in Rheumatology

Important strides have been made in understanding the pathophysiologic basis of many inflammatory conditions in recent years, but rheumatology remains a discipline in which diagnosis is rooted in the medical history skillfully extracted from the patient, the careful physical examination, and the discriminating use of laboratory tests and imaging. Moreover, selection of the most appropriate therapy for patients with rheumatic diseases also remains heavily reliant upon clinical experience. Medical disciplines such as rheumatology that depend significantly upon clinical wisdom are prone to the development of systems of ‘Pearls’ and ‘Myths,’ related to the diseases they call their own, a ‘Pearl’ being a nugget of truth about the diagnosis or treatment of a particular disease that has been gained by dint of clinical experience and a ‘Myth’ being a commonly held belief that influences the practice of many clinicians – but is false. This book will pool together the clinical wisdom of seasoned, expert rheumatologists who participate in the care of patients with autoimmune diseases, systemic inflammatory disorders, and all other rheumatic conditions.

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Histopathology Kidney --Systemic lupus erythematosus

more targeted therapy for psoriatic and rheumatoid arthritis by contrast-enhanced MRI

In the past the treatment for patients with psoriatic arthritis was based on the treatment for those with rheumatoid arthritis
According to a new study ,contrast-enhanced magnetic resonance imaging (MRI) could help to differentiate between psoriatic and rheumatoid arthritis in hands and wrists. the results of the study might lead to a more targeted therapy unique to each condition.

This study that was done at the University Hospital of Tubingen imaged using contrast-enhanced magnetic resonance imaging 31 patients with rheumatoid arthritis and 14 with psoriatic arthritis. The contrast media in rheumatoid and psoriatic arthritis is presumed to be different and the difference can only be seen 15 minutes after the contrast material is given.
“Our study revealed a significant difference in perfusion between those patients with rheumatoid arthritis and psoriatic arthritis after 15 minutes. However, since it was a small group of patients and there was an overlap in perfusion values between both types of arthritis, a diagnosis could not be led by contrast-enhanced MRI alone. Our results are nonetheless promising though,” said Nina Schwenzer, lead author of the study.

Case of Young Man with Henoch-Schönlein Purpura

A 31-year-old Caucasian male is admitted to the hospital with a 10-day history lower extremities rash and hands and feet swelling. He had upper respiratory tract infection before his current symptoms started.

A biopsy of skin lesions at an outside hospital shows ..............

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Purpura and edema of lower extremities

Methotrexate for Rheumatoid Arthritis

A concise video discussing the medication methotrexate for the treatment of inflammatory arthritis such as rheumatoid arthritis