MUST KNOW ABOTH GOUT RHEUMATIC AND SLE.pptx

gout and sle

1. APPROACH TO ARTHRITIS Agus Widiyatmoko

2. Arthritis is an inflammatory process affecting a joint/joints and may present with following symptoms: 1. Pain 2. Stiffness 3. Swelling 4. Limitation of Movement 5. Weakness 6. Fatigue

3. History and Physical Examination Periarticular Bursitis/Tendinitis/Ligament Strain/Bone Pathology Articular Morning stiffness/Warmth/Erythema Non Inflammatory Osteoarthritis CTD Inflammatory Monoarticular Infection/Gout/Pseudogout Oligoarticular AS/Reiter'/Reactive IBD/Psoriatic Polyarticular RA/SLE/ Psoriatic/CTD Absent Present

4. Musculoskeletal Evaluation Articular 1.Symptoms present throughout the range of movement 2. Joint Instability 3. Swelling 4. Presence of deformity Non Articular 1. Symptoms present at a particular point in the range of movement 2. Joint instability absent 3. Swelling absent 4. Deformity absent

5. Articular -Pain both at rest and during motion -Pain worse at rest -Stiffness typically lasts for >30mins -Joint swelling is related to synovial hypertrophy, synovial effusion &/or inflammation of periarticular structures -Limited range of movement -Presence of Warmth and Erythema -Due to alterations in the structure or mechanics of the joint -Pain mainly during motion & improves quickly on rest. -Stiffness not more 15-30 minutes. -Swelling results due to formation of osteophytes or due to soft tissue swelling related to synovial cysts, thickening or effusion. Traumatic Degenerative Mechanical Inflammatory Non inflammatory

6. D/D on the basis of ONSET OF SYMPTOMS Abruptly over few hours to days Trauma Crystal arthritis Septic Arthritis Insidiously over weeks to months Rheumatoid Arthritis Osteoarthritis Seronegative Spondyloarthropathies Chronic Gout

7. D/D on the basis of DURATION OF SYMPTOMS ACUTE, i.e. , <6weeks CHRONIC, i.e. , >6weeks Trauma Juxta-Articular Septic Arthritis Reactive Arthritis Gout Rheumatic Fever Rheumatoid Arthritis SLE Spondyloarthropathies OA Haemochromatosis

8. D/D on the basis of PATTERN OF JOINT INVOLVEMENT Migratory Additive/Simultaneous Intermittent Acute Rheumatic Fever Disseminated Gonococcal Infection Viral Arthritis RA SLE Spondyloarthritids Gout Viral Arthritis Lymes Disease

9. Distribution of affected joints : DIP involved in Psoriatic Arthritis, OA and Gout. Axial Skeleton is involved in AS, especially Lumbar Spine and Sacroiliac Joint. Weight bearing joints e.g. Knee and Hip Joints are especially involved in OA. 1 st Metatarsophalengeal Joint is usually first involved in Gout. Heal Pain due to inflammation at the insertion of Achilles Tendon &/or Plantar Facia is typically seen in Spondyloarthritids.

10. Extra-Articular Manifestations (Constitutional Symptoms) : Presence of Skin, Nail & Mucous Membrane Lesions may points to the possibility of SLE, Psoriatic Arthritis, Scleroderma. Arthritis of IBD may present with the features of Crohns Disease or Ulcerative Colitis. Presence of Urethritis, Conjunctivitis and Arthritis may points to the possibility of Reiter Syndrome that usually follows after non-specific GI or GU Infections.

11. DIAGNOSIS TYPE ADDITIONAL FEATURES LAB & IMAGING OA Noninflammatory, mono/oligo/poly-articular Bone Spurs; knee, hip, PIP, DIP, 1 st MTP, 1 st CMC. Normal ESR/CRP, Osteophytes, Bone Sclerosis Gout Inflammatory, mono/oligo/poly-articular Tophi; Acute attacks f/b spontaneous resolution Raised UA Levels, + UA Crystals in joint fluid, Raised ESR/CRP, Erosions with overhanging borders Pseudogout Inflammatory, mono/oligo/poly-articular Acute/Chronic Attacks Raised ESR/CRP Levels, + CPPD Crystals in joint fluid Septic Joint Inflammatory Monoarticular, rerely Polyarticular Sepsis, Fever Raised ESR/CRP, + Cultures, Leucocytosis, Immunosuppressed RA Inflammatory Polyarticular Extraarticular Manifestations, DIP never Involved Periarticular Osteoporosis, +RF & Anti-CCP, Raised ESR/CRP Pso A. Inflammatory Oligo or Polyarticular Psoriatic skin rash, Asymmetric SI Joint Involvement, Syndesmophytes Erosions, Ankylosis AS Inflammatory Bamboo Spine, Symmetric SI Joint Involvement, Syndesmophytes Ankylosis, Trolly Track Sign, Dagger Sign

12. Target sites of various arthritis in a joint.

13. Common Radiological Features of Arthritis Soft tissue swelling Subchondral sclerosis and erosion Narrowing of joint space Joint effusion. Osteophytes formation Suchondral cystic lesion. Periarticular osteoporosis

14. GOUT - HIPERURICEMIA

15. Pengertian Artritis gout adalah terjadinya peradangan sendi kronis yang disebabkan oleh deposisi monosodium urat (MSU) yang ditandai dengan artritis intermiten yang berhubungan dengan hiperurisemia kronik.

16. Perjalanan alamiah gout terdiri dari empat fase Hiperurisemia asimtomatis, Artritis gout akut, Fase interkritikal atau fase yang diselingi interval tanpa gejala klinis, dan Gout kronis.

17. HIPERURICEMIA ASIMPTOMATIK Kadar asam urat serum > 6,8 mg/dl tanpa ada gejala Penggunaan terapi penurun kadar asam urat pada hiperurisemia tanpa gejala masih kontroversi Terapi : Modifikasi gaya hidup

18. Tahapan Gout Asimptomatik Akut : UA ↑, tanpa keluhan : gejala muncul tiba– tiba dan biasanya menyerang satu atau beberapa persendian. Persendian yang terserang meradang, merah, terasa panas dan bengkak 3. Interkritikal : kelanjutan stadium akut dimana terjadi periode interkritikal asimtomatik. Secara klinik tidak dapat ditemukan tanda-tanda radang akut. 4. Kronis : masa kristal asam urat (tofi) menumpuk di berbagai wilayah jaringan lunak tubuh penderitanya.

19. Gout Akut Onset akut Harus mendapatkan penanganan segera Painful monoarticular arthritis ( Podagra ) MTP atau great toe Pencetus : Perubahan kadar asam urat mendadak Obat-obatan yang meningkatkan kadar asam urat serum

20. Fase Interkritikal dan Gout Kronis Fase Interkritikal 🡪 Periode bebas gejala diantara dua serangan gout akut Solid crystal deposition (tophus) pada persendian (esp. toes, fingers, wrist, knees) dan jaringan (esp. olecranon bursa, pinna, Achilles) ~ sindroma metabolic; CKD; CHF Fase Interkritikal dan gout kronis perlu terapi penurun kadar asam urat dan terapi profilaksis mencegah serangan akut

21. DIAGNOSIS ↑ UA Bukan diagnostic 25% flare ↑ WBC dan LED Gold Standard à Arthrocentesis ( Needle-shaped, negatively briferinget ) Radiografi Erosi dengan overhanging edge ; “ double countor sign ” Terdapat kondisi lain : batu asam urat ; nefropati urat

22. Mikroskopik Needle-shaped negatively briferinget

23. Diet Gout Arthritis Pasien dengan hiperurisemia dan artritis gout direkomendasikan untuk membatasi konsumsi: Alkohol Minuman tinggi fruktosa Makanan tertentu yang tinggi purin (seperti daging merah/ jeroan/produk olahan daging/seafood)

24. Minuman berpemanis Minuman tinggi fruktosa dan minuman ringan berpemanis gula terbukti dapat meningkatkan kadar asam urat serum. Penelitian yang membedakan sumber fruktosa menjadi minuman berpemanis, jus buah dan buah-buahan segar (bukan jus) menunjukkan bahwa minuman berpemanis dan jus buah dapat meningkatkan risiko gout, sedangkan buah-buahan (bukan jus) tidak memiliki risiko yang sama

25. Sayuran tinggi purin Penelitian tentang konsumsi sayuran yang kaya purin (seperti asparagus, bayam, brokoli, dan yang lainnya) ternyata tidak meningkatkan risiko hiperurisemia dan gout. Suatu metaanalisis menemukan bahwa konsumsi sayuran kaya purin tidak berhubungan dengan peningkatan risiko hiperurisemia dan bahkan memiliki efek protektif terhadap gout

26. AKSI MEKANISME OBAT

27. KOMPLIKASI Gout kronik bertophus Kerusakan sendi Nefrolitiasis Nefropati urat

28. PSEUDOGOUT

29. PSEUDOGOUT Calcium pyrophosphate Crystal Deposition Disease (CPPD) is the syndrome secondary to the calcium pyrophosphate in articular tissues. This includes: Chondrocalcinosis, Chronic CPPD and Pseudogout.

30. Pseudogout Etiology: It is unknown but can be secondary to changes in the cartilage matrix or secondary to elevated levels of calcium or inorganic pyrophosphate. Pathology: CPPD crystals are found in the joint capsule and fibrocartilaginous structures. There is neutrophil infiltration and erosions.

31. RHEUMATOID DISEASE

32. INFLAMMATORY ARTHRITIS Rheumatoid Arthritis:- Rheumatoid arthritis is a progressive, chronic, systemic inflammatory disease affecting primarily the synovial joints Onset is usually between 20 and 60 years of age, with the highest incidence among the 40- to 50-year-old group. Under 40 females to male ratio is 3:1 and over 40 equal, 1:1 ratio incidence. The detection of rheumatoid factor, representing specific antibodies in the patient's serum, is an important diagnostic finding

33. Low-grade fever, fatigue, weight loss, muscle soreness, and atrophy. Symmetric peripheral joint pain and swelling, particularly of the hands. Pathologic Features:- Initial synovial inflammation within joints, bursae, and tendon sheaths, with cellular infiltrate, hyperemia, edema,and increased synovial fluid. Synovium becomes hypertrophied to form granulation tissue (pannus), which spreads over cartilage surface. At the bare areas pannus directly invades into the bone, resulting in marginal erosions and cartilage destruction. A rheumatoid nodule is diagnostic and consists of three distinct zones: fibrinoid degeneration and necrosis (central), radial palisading of fibroblasts (middle), and fibrous tissue with small cell infiltrate (outer).

34. Boutonniere Deformity Swan Neck Deformity

35. Kriteria Diagnosis

36. Pemeriksaan Penunjang

37. Radiologic Features Early radiographic changes are most commonly seen in the hands and feet. Bilateral and symmetric distribution, periarticular soft tissue swelling(these are typically the first radiographic signs of rheumatoid arthritis.), juxta-articular osteoporosis, juxta-articular solid or laminated periostitis, marginal erosions and cysts, and uniform loss of joint space. Later, radiographic changes may be seen, including marked deformities with subluxation, dislocation, articular bony destruction, bony fusion, and complete destruction of joint space. Hand: earliest changes are seen at the metacarpophalangeal and PIP joints. Evaluation should include the semisupination view of the hands (Norgaard projection) for marginal erosions on metacarpal heads and deformities like ulnar deviation, boutonniere, swan neck, spindle digit.

38. Wrist: earliest change is erosion of ulnar styloid, multiple carpal erosions (spotty carpal sign), most common location for bony ankylosis, carpal radial rotation, zigzag deformity, Terry Thomas’ sign. Feet: earliest changes seen at the fourth and fifth metatarsal phalangeal joints. Changes parallel and are identical to that seen in the hands; Lanois deformity—dorsal subluxation of the metatarsal-phalangeal joints, with fibular deviation. Cervical spine: most commonly affected area of the spine; involved in up to 70% of rheumatoid patients. Increased atlantodental interspace > 3 mm (especially in flexion), odontoid erosions, subluxations (especially C3, C4, and C5). Narrowed intervertebral discs, apophyseal joints show erosions and narrowed joint space and may ankylose. Tapered spinous processes and generalized osteoporosis. Hips: uniform loss of joint space (axial migration), minimal erosions, protrusio acetabuli (most common cause),particularly bilaterally. Knees: uniform loss of joint space, marginal erosions (particularly at the tibial condyles), and osteoporosis; often associated with large Baker’s cysts.

39. Anteroposterior (A) and lateral (B) radiographs of the knee shows periarticular osteoporosis, joint effusion, and lack of osteophytosis.

40. Anteroposterior radiograph of the right hip shows erosions of the femoral head and acetabulum, concentric narrowing of the hip joint, and acetabular protrusio.

41. (A) Lateral radiograph of the foot of shows fluid in the retrocalcaneal bursa (arrow) associated with erosion of the calcaneus (curved arrow). MRI demonstrates bone erosion in the posterior process of the calcaneus arrowhead) associated with extensive surrounding bone marrow edema and retrocalcaneal and retro-Achilles bursitis (arrows).

42. Xray demonstrates erosions in the radiocarpal and intercarpal articulations as well as the carpometacarpal joint, bilaterally (open arrows). Note, in addition, subtle erosions of the head of the first, third, fourth, and fifth metacarpals of the left hand and of the head of the second metacarpal of the right hand (arrows). A small erosion at the base of the middle phalanx of the ring finger of the left hand (arrowheads) and the erosion in the right triquetropisiform joint (curved arrow) are also well seen.

43. Oblique radiograph of the hand shows the swan neck deformity of the second through fifth fingers

44. Radiograph of the hands demonstrates the boutonnière deformity in the small and ring fingers of the right hand and in the ring finger of the left hand

45. Radiograph of the hands demonstrates the main-en-lorgnette deformity- the telescoping the fingers secondary to destructive joint changes and dislocations in the metacarpophalangeal joints

46. Radiograph of the cervical spine

47. Juvenile rheumatoid arthritis Chronic polyarthritis resembling rheumatoid arthritis clinically and histologically beginning before 16 years of age Synonyms include Still’s disease and juvenile chronic arthritis. More common in females < 16 years, with peak incidence at 2-5 and 9-12 years.

48. TYPES Adult form (seropositive) Poorest prognosis Seronegative form:- Classic systemic ,Polyarticular Pauciarticular-monoarticular Distinct lack of rheumatoid factor Symptoms include fever, characteristic rash, lymphadenopathy, iridocyclitis (especially in monoarticularforms), no subcutaneous nodules, and growth disturbance. Distinct lack of rheumatoid arthritis

49. Radiologic Features General features include soft tissue swelling, osteoporosis, periostitis, growth disturbances, ankylosis, loss of joint space, erosions, subluxations, and epiphyseal compression fractures. Target sites include cervical spine, hands, feet, knees, and hips. Cervical spine: atlantoaxial dislocations, hypoplastic C2-C4 vertebral bodies and discs with ankylosed apophyseal joints. Tarsal and carpal ankylosis common. Growth deformities: brachydactyly, ballooned epiphyses, squashed carpi, and squared patellae.

50. Lateral Lumbar Note that osteoporosis and compression fractures have produced a biconcave appearance of the endplates. Lateral Cervical. Observe the vertebral body hypoplasia of the second, third, fourth, and fifth segments. The odontoid appears enlarged. C. Lateral Cervical. Note that the vertebral bodies are hypoplastic in combination with posterior joint ankylosis. These are characteristic cervical spine changes

51. Radiograph of both hands shows destructive changes in the metacarpophalangeal and interphalangeal joints. Note also joints ankylosis in both wrists. the periarticular soft tissue swelling and periostitis (arrows)

52. Radiograph of both knees of a 20- year-old woman shows overgrowth of the medial condyles, one of the characteristic features of this disorder

53. Ankylosing Spondylitis A chronic inflammatory disorder principally affecting the articulations, ligaments, and tendons of the spine and pelvis, often resulting in complete polyarticular ankylosis. Synonyms include Marie-Strumpell disease, rhizomelic spondylitis, pelvospondylitis ossificans, and rheumatoid spondylitis. Onset is usually between 15 and 35 years and involves males 10:1. Initiates at the sacroiliac joints bilaterally, then ascends the spine. Pain and tenderness, especially over bony protuberances, and increasing stiffness and sciatica is often bilateral or may alternate from side to side. Complications include iritis, aortitis, valvular incompetence, aneurysms, conduction blocks, upper lobe pulmonary fibrosis, inflammatory bowel disease, renal failure owing to secondary amyloidosis, carrot-stick fractures, Andersson’s lesion, and prosthesis ankylosis. The most commonly involved areas are the sacroiliac joints, spine, and proximal large joints of the shoulder, hip, and rib cage.

54. Pathologic Features In synovial joints, the initial change is that of a non- specific synovitis similar to rheumatoid arthritis, except that it is less extensive and of lower intensity (pannus formation), with subsequent fibroplasia and cartilaginous etaplasia, leading to resultant ossification. In cartilage joints, the initial subchondral osteitis is replaced by fibrous tissue that subsequently ossifies. In the outer annulus fibers this forms syndesmophytes. At entheses, inflammatory changes at ligamentous attachments result in bony erosions, sclerosis, and periostitis.

55. Radiologic Features

56. Lateral radiograph of the lumbar spine demonstrates squaring of the vertebral bodies secondary to small osseous erosions at the corners. This finding is an early radiographic feature of ankylosing spondylitis. Note also the formation of syndesmophytes at the L4- 5 disk space.

57. Lateral radiograph of the cervical spine in a shows anterior syndesmophytes bridging the vertebral bodies and posterior f usion of the apophyseal joints, together with paravertebral ossifications, producing a “bamboo-spine” appearance. radiograph the fusion of the sacroiliac joints and the involvement of both hip joints, which show axial migration of the femoral heads (D)MRI shows anterior syndesmophytes, calcification of the posterior longitudinal ligament, and preservation of the intervertebral disks.

58. A lateral radiograph of the lower lumbar spine of shows early inflammatory changes manifesting by so-called shiny corners (Romanus lesion) (arrowheads) and squaring of the vertebral bodies (arrows). T2-weighted MRI in a 26-year-old man shows early signs of ankylosing spondylitis of the lumbar spine, the shiny corners (arrows). T2-weighted MRI of the sacroiliac joints in the same patient demonstrates bone marrow edema adjacent to the sacroiliac joints and erosive changes bilaterally, more prominent on the left (arrows).

59. AP Sacrum. Note that bilateral sacroiliitis is clearly seen with erosions, hazy joint margin, and subchondral iliac sclerosis (arrows). Axial CT: Sacroiliac Joints. Observe the erosive iliac lesions (arrows) and the subchondral sclerosis arrowheads).

60. Psoriatic Arthritis Psoriasis is a common skin disorder associated with joint disease and characterized by peripheral joint destruction and deformity: Age 20-50 years with male and female equally affected. Arthritis is usually in peripheral joints, especially DIP joints. Soft tissue findings: fusiform soft tissue swelling around the joints which can progress so that whole digit is swollen (sausage digit or dactylitis) Marginal erosions also often show fluffy periostitis from new bone formation

61. Radiologic Features General features include soft tissue swelling, normal bone mineralization, erosions, and tapered bone ends, prominent juxta- articular fluffy periostitis, and joint-space widening or bony ankylosis. Hands and feet: asymmetric involvement and ray pattern, most commonly involves DIP joints, no osteoporosis, mouse ears sign, widened joint space owing to fibrous tissue deposition and bone resorption, pencil-in-cup deformity, opera glass hand deformity, no ulnar deviation. Sacroiliac joint: involved in up to 50% of psoriatic arthritis patients, usually bilateral but asymmetric and unusual to be narrowed and ankylosed. Spine: atlantoaxial subluxation and dislocation, normal apophyseal joints (except in the cervical spine),syndesmophytes of two types— non—marginal, marginal (non-marginal are the most common)— broad-based and tapered, asymmetric, unilateral, and most common in the upper lumbar and lower thoracic spine.

62. RAY PATTERN PA Hand. Note the erosive changes are present at the three joints of the second digit (arrows). This pattern of arthritis is virtually diagnostic of psoriasis

63. Pencil and cup deformity Pencilling

64. Early Distal Interphalangeal Joint Changes. Note that erosions (arrows), periostitis (arrowheads), and soft tissue swelling characterize the earliest abnormalities Combination of erosions and fluffy periostitis produces the mouse ears appearance in psoriasis. MOUSE EAR SIGN

65. Non- Marginal Syndesmophyte. Note the thick, vertical ossifications that arise just beyond the vertebral body margins (arrows).

66. Oblique radiograph of the lumbar spine in a shows a characteristic single coarse syndesmophyte bridging the bodies of L3 and L4. The right sacroiliac joint is also affected. (B) AP radiograph of the lumbar spine with psoriasis reveals paraspinal ossification at the level of L2-3.

67. PA Hand. Fluffy and Linear. Note that close to the joint near the site of articular erosion, the periosteal new bone is typically fluffy arrowheads). Farther down the shaft a linear pattern may be seen (arrow). Great Toe: Fluffy. Note that adjacent to the erosions a fluffy and irregular type of periostitis can be seen arrowheads). The entire distal phalanx is sclerotic, a reliable sign of psoriatic arthritis involving the great toe.

68. Note severe joint destruction, especially at the metatarsophalangeal articulations, has resulted in fibular deviation and dorsal dislocation of the digits (Lanois’ deformity). The presence of a pencil- in-cup deformity (arrow) at the interphalangeal joint of the big toe and osseous ankylosis of the first metatarsophalangeal and second and third proximal interphalangeal articulations (arrowheads) makes the diagnosis of psoriatic arthritis most likely ARTHRITIS MUTILANS

69. DIFFERENTIAL DIAGNOSIS Rheumatoid arthritis there is a MCP joint predominance in rheumatoid arthritis (RA) vs interphalangeal predominant distribution in PsA bone proliferation not a feature in RA osteoporosis not a feature in PsA Erosive osteoarthritis gull wing” central erosions are present in erosive OA vs “mouse ears” peripheral bare area erosions in PsA reactive arthritis (Reiter syndrome) “tends to involve feet > hands

70. REITER’S SYNDROME A triad of urethritis, conjunctivitis, and polyarthritis, usually following sexual exposure or, less commonly, certain types of dysentery. It typically occurs between the ages of 18 and 40, and is as much as 50 times more prevalent in males Joint symptoms typically consist of an asymmetric painful effusion, especially of the lower extremity Pain at the plantar or Achilles calcaneal attachment (lover’s heels) in a young male patient should suggest the diagnosis. These joint symptoms are of short duration and self-limiting within 2-3 months, but recurrences are common.

71. Radiologic Features Swelling, osteoporosis, uniform loss of joint space, erosions, periostitis. Specific target sites: forefoot, calcaneum, ankle, knee, sacroiliac, spine. Foot: metatarsophalangeal and interphalangeal joints. Dorsal subluxation of the proximal phalanges and fibular deviation of the digits results in the Lanois deformity. Calcaneum: plantar and Achilles insertions. Ankle: loss of joint space, swelling, periostitis. Sacroiliac: erosions, sclerosis, loss of joint margin, asymmetric involvement and often unilateral. Spine: thoracolumbar, asymmetric, skip non-marginal syndesmophytes and, rarely, atlantoaxial instability Knee: the only change usually visible at the knee is effusion and, occasionally, periostitis of the distal femoral metaphysis. A Pellegrini- Stieda type calcification of the medial collateral ligament may be seen

72. Xray foot shows the thin layer of periosteal new bone at the phalangeal base at the third metatarsophalangeal joint (arrows). There is also a notable diminished density in the metatarsal head (arrowhead).

73. Xray Finger show marginal erosions (arrows), linear periostitis (arrowheads), and soft tissue swelling (crossed arrows) at the proximal interphalangeal joint.

74. CALCANEUS. A. Early Erosive Changes: Achilles Tendon. Shows small lucent defects (arrows) and adjacent periostitis (arrowhead). B. Pathophysiology. The inflamed pre-Achilles bursa (arrowheads) becomes the site for pannus formation and subsequent subperiosteal resorption of the adjacent calcaneus (arrow). C. Advanced Erosive Changes. Note that the lucent defects are larger (arrows), with prominent periostitis (arrowheads). Note the fluffy calcaneal spur owing to inflammatory enthesopathy (crossed arrow).

75. MEDIAL COLLATERAL LIGAMENT CALCIFICATION. Note the irregular linear density adjacent to the medial epicondyle (arrow). This is a Pellegrini-Stieda type of calcification within the medial collateral ligament and may be seen in approximately 10% of Reiter’s syndrome patients

76. AP radiograph of the lumbar spine with reactive arthritis demonstrates a paraspinal ossification bridging the L2 and L3 vertebrae.

77. Scleroderma Systemic inflammatory connective tissue disease affecting the skin, lungs, gastrointestinal tract, heart, kidneys, and musculoskeletal system More common in females 30-50 years of age. Initial peripheral pain and swelling, with high incidence of Raynaud’s phenomenon. The pathological features are low-grade perivascular inflammation with atrophy and fibrosis of adjacent collagen.

78. Radological features Hand is most commonly involved Soft tissue:-tapered, conical fingertips ,retraction of fingertip,loss of overlying skin folds ,calcification: skin (calcinosis cutis) intra-articular. Bone:-Resorption—distal tufts(acroosteolysis) Joint:-Erosive arthropathy at first metacarpal-carpal joint

79. SCLERODERM A WITH DIGITAL SKIN RETRACTION AND EARLY ACROOSTEOL YSIS. Note the atrophy and retraction of the soft tissues of the fingertip at the fourth digit (arrows). Resorption of the distal tuft is also seen (arrowhead). The combination of these two findings is highly indicative of scleroderma.

80. SCLERODERMA: DIGITAL PATTERNS OF CALCINOSIS CUTIS. A. Punctate. B. Sheet- Like .

81. Soft Tissue Rheumatism -Most common cause of Musculo-Skeletal Pain. -Mostly associated with Fibromyalgia. -Characterised by Bursitis, tendonitis or tenosynovitis. -Improves with Local Steroid Injections.

82. Polymyalgia Rheumatica(PMR) Presents in elderly males as proximal limb girdle pain, morning stiffness and constitutional symptoms. Associated with Temporal Arteritis(TA) in 40% of cases. Patients with TA presents with headache, scalp tenderness, jaw & tongue claudication, vision disturbances and stroke. PMR : Elevated ESR TA : Elevated ESR (often >100mm/hr.)

83. SYSTEMIC LUPUS ERYTHEMATOSUS KENALI, CEGAH dan TERAPI Agus Widiyatmoko

84. PENGERTIAN Lupus eritematosus sistemik (LES) adalah gangguan autoimun yang ditandai dengan antibodi terhadap antigen nuklir dan sitoplasma, peradangan multisistem, dan perjalanan penyakit yang kambuh dan hilang. Faktor gen dan lingkungan berperan pada patofisiologi penyakit ini Lebih dari 90% kasus SLE terjadi pada wanita, seringkali dimulai pada usia subur.

85. PATHOPHYSIOLOGY

86. Patofisiologi lanjutan Limfosit mengalami disregulasi (tidak toleran) menargetkan antigen intraseluler yang biasanya dilindungi oleh limfosit 🡪 membentuk kompleks imun Sel T telah lama dianggap memainkan peran sentral dalam patogenesis SLE, dan sel T dari pasien dengan lupus menunjukkan cacat pada fungsi sinyal dan efektor. Sel T sitotoksik dan sel T supresor (yang biasanya akan menurunkan regulasi respons imun) menurun. Sel T helper (CD4+) meningkat.

87. Patofisiologi Antibodi antinuklear serum (ANAs) ditemukan di hampir semua individu dengan SLE aktif. Antibodi terhadap DNA untai ganda (dsDNA) relatif spesifik untuk diagnosis SLE.

88. ETIOLOGI Sebuah studi berbasis populasi dari Denmark menemukan bahwa rasio SLE adalah 10,3 kali pada anggota keluarga tingkat pertama pada pasien dengan SLE, dan 3,6 kali pada keluarga tingkat kedua atau ketiga. Studi antigen leukosit (HLA) mengungkapkan bahwa HLA-A1, HLA-B8, dan HLA-DR3 lebih sering terjadi pada orang dengan SLE daripada pada populasi umum. Sejumlah penelitian menemukan peran infeksi yang juga dapat memicu autoimunitas.

89. Etiologi Infeksi Pasien dengan SLE memiliki titer antibodi yang lebih tinggi terhadap virus Epstein-Barr (EBV) Sebuah studi oleh Manfredo Vieira et al menemukan bahwa pada strain tikus yang autoimun, translokasi pathobio usus, Enterococcus gallinarum, ke hati dan jaringan sistemik lainnya (memicu hilangnya integritas barrier usus) memicu reaksi autoimun

90. ETIOLOGI LINGKUNGAN Penyebab SLE yang berhubungan dengan lingkungan dan paparan kurang jelas. Kemungkinan faktor risiko awal kehidupan sebagai berikut: Berat badan lahir rendah (< 2.500 g) Kelahiran prematur (1 bulan lebih awal) Paparan pestisida pertanian pada anak-anak

91. Faktor potensial lainnya termasuk yang berikut: Debu silika dan merokok dapat meningkatkan risiko terkena SLE Penggunaan estrogen pada wanita pascamenopause meningkatkan risiko mengembangkan SLE. Fotosensitifitas jelas merupakan pencetus SLE Menyusui dikaitkan dengan penurunan risiko pengembangan SLE Data menunjukkan bahwa hasil kehamilan menguntungkan dan flare jarang terjadi di antara pasien dengan SLE ringan-sedang yang tidak aktif atau stabil. Defisiensi vitamin D dapat memicu autoimunitas dan perkembangan penyakit rematik, termasuk SLE. Ras ASIA memiliki risiko SLE lebih tinggi Faktor potensial lainnya termasuk yang berikut: Faktor potensial lainnya termasuk yang berikut:

92. KEWASPADAAN LES Manifestasi klinis yang beragam kadang menyulitkan diagnosis LES LES perlu diwaspadai pada perempuan muda yang memiliki manifestasi klinis lebih dari 2 sistem organ atau lebih Setiap pasien yang dicurigai LES perlu pemeriksaan lanjutan untuk menegakkan diagnosis

93. DIAGNOSIS LES (ACR)

94. TERAPI LES

95. Three Pillars of Therapy Education and counseling Exercise / medical rehabilitation program. Pharmacologic therapy: NSAIDs Steroid Anti-malaria Imunosupressive / cytotoxic drugs ESCISIT. Ann Rheum Dis. 2008;67:195–205 IRA. Panduan Diagnosis dan Penatalaksanaan SLE. 2003

96. Key Points on Education Wallace DJ. Duboi’s Lupus Erythematosus 2007:1133 Information about what lupus is and the causes. Type of disease and its characteristics. . Physical related problems: The use of exercises in conjunction with OP prevention due to steroid ADE, rest, assistive device, diet, infection and contraception. Psychological related problems: Insight of the patients with lupus, how to cope with stress, fatigue, psychological trauma, family or work related problems, alleviating pain.

97. Key Message on Education Wallace DJ. Duboi’s Lupus Erythematosus 2007:1133 Drugs use: Dosage, type of drugs, duration of treatment. Supplement Resource information includes patient education materials, counseling availability, self-help, websites. Is there any supporting groups? Lupus foundations?

98. Medical Rehabilitation for Lupus IRA. Panduan Diagnosis dan Penatalaksanaan SLE. 2003 Rest: ask the patients to stay active. Physical therapy and exercises (muscle strengthening exercise, ROM exercise). Therapy with modalities. Orthotic.

99. Management of SLE

100. Renal, NPSLE Cyc Micophenolate Disease severity Mild Moderate Skin, joint, serositis Immune thrombocytopenia AIHA Severe HC AZA or MTX Cs-A Tacrolimus Skin, joint, serositis, vasculitis AZA or MTX Cs-A Tacrolimus Micophenolate (+/- Cs-A) MTX (+FA) or Cs-A IVIG Cs-A Tacrolimus MS/MMF (+/- Cs-A) Cyc Skin, joint Flow chart of suggested drug treatment of SLE Morton SJ, Powell RJ. Clin Exp Allergy. 2001;31:686-93

101. NEW HOPE IN SLE

102. NEW TREATMENT?

103. THE FUTURE MSC for treatment in SLE Targeting sel T reg to reduced systemic inflammation in SLE IL-10 to regulated inflammation in SLE

104. ALHAMDULILLAH