Crystals associated diseases 3

. TUBERCULOSIS

Caused by bacteria belonging to the Mycobacterium tuberculosis complex The disease usually affects the lungs, although in up to one-third of cases other organs are involved Transmission usually takes place through the air borne spread of droplet nuclei produced by patients with active pulmonary tuberculosis

T.B



The bacillus causing tuberculosis, Mycobacterium tuberculosis, was identified and described on 1882 by Robert Koch. He received the Nobel Prize in 1905 for this discovery



ETIOLOGIC AGENT the most frequent and important agentof human disease is M. tuberculosis . The complex includes: M. bovis the bovine tubercle bacillus, (the cause of a small percentage of cases in developing countries), M. africanum M. microti M. canettii

. the extrapulmonary sites most commonly involved in tuberculosis are; pleura, the lymph nodes genitourinary tract, bones ,joints, meninges, peritoneum,and pericardium

TB OF ELBOW JOINT

TB OF ELBOW

. SKELETAL TUBERCULOSIS the pathogenesis is related toa- reactivation of hematogenous foci b- spread from adjacent paravertebral lymph nodes: 1-Weight-bearing joints (spine, hips, and knees—in that order) are affected most commonly. 2-Spinal tuberculosis (Pott’s disease or tuberculous spondylitis); often involves two or more adjacent vertebral bodies.

TB SPINE

the upper thoracic spine is the most common site of spinal tuberculosis in children, the lower thoracic and upper lumbar vertebrae are usually affected in adults. With advanced disease, collapse of vertebral bodies results in kyphosis (gibbus).

A paravertebral “cold” abscess may also form In the upper spine, this abscess may track to the chest wall as a mass; in the lower spine, it may reach the inguinal ligaments or present as a psoas abscess. -(CT) or (MRI) reveals the characteristic lesion and suggests its etiology,

Aspiration of the abscess or bone biopsy confirms the tuberculous etiology, as cultures are usually positive and histologic findings are highly typical. -a catastrophic complication of Pott’s disease is paraplegia, which is usually due to an abscess or a lesion compressing the spinal cord. Paraparesis due to a large abscess is a medical emergency and requires abscess drainage.

Exrtapulmonary TB

Mx Skeletal tuberculosis responds to chemotherapy, but severe cases may require surgery. the diagnosis is first suspected by chest radiograph it shows the typical picture of upper lobe infiltrates with cavitation

A presumptive diagnosis is commonly based on the finding of AFB on microscopic examination of a diagnostic specimen such as 1-a smear of expectorated sputum 2-or of tissue (for example, a lymph node biopsy).

Definitive diagnosis depends on the isolationand identification ofM. tuberculosis from a diagnostic specimen . 4 to 8 weeks may be required before growthis detected.

TREATMENTShort-course regimens are divided into an initial, or bactericidal, phase and a continuation, or sterilizing, phase. During the initial phase, the majority of the tubercle bacilli are killed, symptomsresolve, and the patient becomes noninfectious. The continuation phase is required to eliminate persisting mycobacteria and prevent relapse.-

The treatment regimen of choice for virtually all forms of tuberculosis consists of a 2-month initial phase OF Isoniazid, Rifampin, Pyrazinamide, and Ethambutol (4*2) FOLLOWED BY 4 month continuation phase of Isoniazid and Rifampin .(2*4)Treatment may be given daily or intermittently

extrapulmonary tuberculosis -can be treated with the 6 month regimen recommended for patients with pulmonary disease

VIRAL ARTHRITIS

are self-limiting
The usual presentation is with acute polyarthritis, fever or viral prodrome and rash.
Diagnosis is confirmed by a rise in specific IgM.
Polyarthritis may also rarely occur with hepatitis B and C, rubella and HIV infection.


Psoriatic arthritis (PsA)

Psoriatic arthritis (PsA)

occurs in 7–20% of patients with psoriasis and in up to 0.6% of the general population.
The onset is usually between 25 and 40 years of age.
Most patients (70%) have pre-existing psoriasis but in 20% the arthritis predates the occurrence of skin disease.
Occasionally, the arthritis and psoriasis develop synchronously..

Clinical features

The presentation is with pain and swelling affecting the joints and entheses.
Several patterns of joint involvement are recognised but the course is generally one of intermittent exacerbation followed by varying periods of complete or near-complete remission.
Destructive arthritis and disability are uncommon, except in the case of arthritis mutilans

Types of joints involvement

1-Asymmetrical inflammatory oligoarthritis (sero-ve like)
affects about 40% of patients and often presents abruptly .
This occurs most characteristically in the hands and feet, when synovitis of a finger or toeis coupled with tenosynovitis, enthesitis and inflammation of intervening tissue to give a‘sausage digit’ or dactylitis .

Large joints, such as the knee and ankle, may also be

involved, sometimes with very large effusions.


Types of joints involvement
2-Symmetrical polyarthritis (RA)
occurs in about 25% of cases. It predominates in women and may strongly resemble RA, with symmetrical involvement of small and large joints in both upper and lower limbs.
Nodules and other extra-articular features
of RA are absent and arthritis is generally less
extensive and more benign. Much of the hand
deformity often results from tenosynovitis and soft
tissue contractures(how to differentiate)

Types of joints involvement

3-Psoriatic spondylitis (AS)like
presents a similar clinical picture to AS but with less severe involvement.

It may occur alone or with any of the other clinical patterns described above and is typically unilateral or asymmetric in severity.

Types of joints involvement

4-Distal IPJ arthritis (the typical type)
is an uncommon but characteristic pattern affecting men more often than women. It
targets finger DIP joints and surrounding
periarticular tissues, almost invariably with
accompanying nail dystrophy.


Types of joints involvement
5-Arthritis mutilans (occurs in 5% of cases of PsA)

is a deforming erosive arthritis

targeting the fingers and toes that Prominent cartilage and bone destruction results in marked instability.
The encasing skin appears invaginated and ‘telescoped’ (‘main en lorgnette’) and the finger can be pulled back to its original length

Arthritis mutilans

Nail changes include

pitting, onycholysis, subungual hyperkeratosis and horizontal ridging.
They are found in 85% of those with PsA and only 30% of those with uncomplicated psoriasis, and can occur in the absence of skin disease.














The characteristic rash of psoriasis may be widespread, or confined to the scalp, natal cleft and umbilicus, where it is easily overlooked.
Conjunctivitis can occur, whereas uveitis is mainly confined
to HLA-B27-positive individuals with sacroiliitis
and spondylitis

Investigations

The diagnosis is made on clinical grounds. Autoantibodies are generally negative and acute phase reactants,
such as ESR and CRP, are raised in only a proportion of patients with active disease

Investigations

X-rays may be normal or
show erosive change with joint space narrowing. Features that favour PsA over RA include the characteristic distribution of proliferative erosions with marked new bone formation, absence of periarticular osteoporosis and osteosclerosis

Investigations

MRI and ultrasound with power Doppler are increasingly employed to detect synovial inflammation and inflammation at the entheses.


Management
NSAID and analgesics may be sufficient
to control symptoms in mild disease.

Intra-articular steroid injections can control synovitis.

Management
Splints and prolonged rest should be avoided because of the tendency to fibrous and bony ankylosis.

Patients with spondylitis should be prescribed the same exercise and posture regime as in AS.

Management

Therapy with DMARDs should be considered for persistent synovitis unresponsive to conservative treatment.

Methotrexate is the drug of first choice since it may also help skin psoriasis

Management
but other DMARDs may also be effective, including sulfasalazine, ciclosporin and
leflunomide.

Hydroxychloroquine is generally avoided, as it can cause exfoliative skin reactions.

Management
Anti-TNF treatment for patients with active synovitis who respond inadequately to standard DMARDs.
This is effective for both PsA and psoriasis.
Other biological treatments, such as ustekinumab, are emerging, which target the IL-12/23 receptor.
Ustekinumab is highly effective in the treatment of psoriatic skin disease and is often effectve in PsA

JUVENILE IDIOPATHIC ARTHRITIS

JIA (also JCA.JRA)is the most common form of childhood arthritis.-it is of unknown etiology.Prevalence 57-220 per 100 000 Of childern younger than 16 years.50 % has active disease that persist into adulthood.

To Dx JIA arthritis must be present for at least 6/52There is no specific test - The Dx is by exclusion

JIA is subdivided into many categories

1-Systemic(Still s) 2-17 %2-Polyarticular RF+ve 2-10 %3-Polyarticular RF-ve 10-28%4-Oligoarthritis (persistant) 12-30%5-Oligoarticular (extended) 12-30%6-Enthesitis related arthritis 3 -10%7-Psoriatic arthritis 2-10%8-Undifferentiated 2-20%%

Systemic (or Still s disease) it was discribed by Still in 1897 -arthritis + fever- NON pruritic rash-generalized LAP-HSM-serositis-M:F 1:1-leukocytosis-vasculitis is a complication - uveitis is rare

OLIGOARTHRITIS-arthritis < 4 joints-persistent and extended F:M 4:1 -PERSISTENT carries high risk of uveitis 30-50% -persistent remission rate 40-70%-extended started in < 4 j/the first 6 months then involves > 5 joints

ANA +ve females with persistent oligoarticular JIA are at high risk of uveitis (30-50%) and slit lamp eye examination is recommended


UVEITIS



PSORIATIC ASSOCIATED JIA-arthritis + psoriasis in patient < 16 years old- only 10% of patients has the onset of psoriatic rash and arthritis at the same time-nail finding is evident (pitting,onycholysis& dactilitis)-uveitis risk is 20%

psoriasis

Nail changes in psoriasis

ENTHESITIS RELATED ARTHRITIS-arthritis-enthesitis-sacroiliac j involvement-+ve HLA-B27-uveitis up to 25%-enthesitis is inflammation of tendon insertion,ligaments or fascia insertion into the bone-aortic involvement may occur

Enthesitis

Inflammatoty arthritis in children is much less common than in adult . there is no specific test which definitely prove the diagnosis.Etiology is unknown.The most recent classification is the international league rheumatism(ILAR)

JRA POLYARTICULAR

JRA

KNEE EFFUSION

PROGNOSIS

The disease is active in up to 35 % of children after 10 years follow up and 30% have erosive arthritis .Reported remission rate for JIA in 5-10 year follow up studies confirm the differential between oligoarticular (40-80%) polyarticular (20-54% ) and systemic(0-35% in remission)JIA.

A MULTIDISCIPLINARY APPROACH IS RECOMMENDED .which involve;rheumatologist , paediatricians, ophthalmologist,dentist,psychologists, physiotherapist, occupational therapist, nurses, social workers and teachers.


Physiotherapy will reduce deformity.Rest is indicated when the joints are acuetly inflammed.splints to prevent flexion deformityHydrotherapy in warm pool is particularly useful.The child should be kept active and ambulant.

NSAID are used at higher doses than in adults (approximately 10-20mg/Kg/day of naproxine).Aspirin should be avoided because of the risk of Reye s syndrome .Intra articular steroid injections are used to treat resistant joints ..

Methotrixate is added for at least 2 years .-MTX& Sulphasalazine are helpful for polyarticular JIA.Combination therapy is used for resistant cases.

Long term corticosteroids are indicated for 1-severe systemic disease (Still s) 2-and for chronic uveitis not responding to local therapy

Advanced therapybiologic agents like (TNF-ALPHA inhibitors) Etanercept,Infliximab,Adalimumab are more useful for polyarticular than systemic type.they are now used with excellent response in severe JIA and in uveitis and vasculitis..

-Anti (Interleuken-1)IL-1 ( Anakinra ) & Anti IL-6 (Tocilizumab) are used for systemic onset type

Surgery like total hip replacement is indicated for children with severe hip involvement

Poor prognosis1-polyarticular disease.2-systemic arthritis that evolve into polyarthritis.3-extended oligiarticular disease.4- polyarticular disease attributable to psoriatic arthritis.

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