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Scleromalasi (perforans) ved RA

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Presentation on theme: "Scleromalasi (perforans) ved RA"— Presentation transcript:

1 Scleromalasi (perforans) ved RA
Fig Sclerosing keratitis (white corneal lesion) associated with active necrotizing scleritis (upper quadrant). Scleromalasi (perforans) ved RA

2 Sclerodaktyli

3 CREST : calcinosis, Raynaud's phenomenon, "eosophageal" dysmotility ,
sclerodactyly, and telangectasia

4 Scleroderma species - potetrøyksopp
                                                           Scleroderma species - potetrøyksopp

5 ”Slapped cheek" disease, a.k.a. fifth disease due to B19 parvovirus
Other clinical syndromes include arthritis (especially in young women) and aplastic crisis in chronic hemolytic anemia

6 Rheumatoide noduli (knuter)

7 CCLE: kronisk kutan LE (=DLE) - etterlater arr !
A 52 year old woman with a long history of photosensitivity and facial skin rashes treated by a dermatologist presented with proteinuria and facial skin lesions in a butterfly distribution as shown in the picture. The differential diagnosis consists of: 1. Acute butterfly skin rash of lupus erythematosus    2. Atrophic skin changes due to local corticosteroid treatment  Wrong answer 3. Chronic lupus erythematosus with a butterfly appearance   4. Combination of skin lesion due to chronic lupus erythematosus and local treatment with corticosteroids. CCLE: kronisk kutan LE (=DLE) - etterlater arr !

8 Hypergammaglobulimeniske purpura ved prim Sjøgren (cf Waldenstrøm)
A 58 year-old woman with a history of Sjögren’s syndrome (SS) for the last 10 years presented to Rheumatology Clinic with a skin rash involving the lower extremities. Clinical examination revealed bilateral parotid gland enlargement and palpable purpura over the extensor surfaces of the lower extremities. Antinuclear antibodies (ANA) were 1/1280 with a fine speckled pattern, IgM rheumatoid factor (RF) was 1/320 and antibodies to Ro (SSA) and La (SSB) were also positive. The differential diagnosis for the palpable purpura in this patient comprises: 1. Hypergammaglobulinemic purpura 2. Idiopathic thrombocytopenic purpura  Wrong answer 3. Waldenström macroglobulinemia   4. T-cell lymphoma   Hypergammaglobulimeniske purpura ved prim Sjøgren (cf Waldenstrøm)

9 Fig. 8.7 Tendon. (a) Micrograph of tendon showing the organized bundles of collagen. (b) Tendon insertion showing a population of chondrocytes at the bone–tendon interface and the collagen fibers crossing the interface. (Courtesy of Dr John Clark.) Enthesis

10 ” sunrise view ”

11 Sacroileitis Ankylosis

12 Fig The red hot joint. Septic arthritis of the ring finger metacarpophalangeal joint showing swelling and intense redness of the skin. Septisk artritt

13 Staphylococcus aureus septic arthritis of the left hip
Staphylococcus aureus septic arthritis of the left hip. The patient presented with left hip pain and fever. The initial film was normal 6 weeks later there is periarticular osteoporosis and cortical destruction of the femoral head.

14 Wegener’s granulomatosis

15 A 55 year old woman with a history of “bronchial asthma”, recurrent iritis and articular chondritis presented with a high fever. Clinical examination revealed a dyspnoiec patient with a moon face and a saddle-shaped nose with diffuse wheezing bilaterally over the lungs. Laboratory evaluation showed leukocytosis with polymorphonuclearcytosis, high ESR, high CRP, and negative ANCA. The chest X-ray was normal. The differential diagnosis consists of: 1. Relapsing polychondritis 2. Cogan’s syndrome   3. Wegener's granulomatosis  Wrong answer 4. Systemic vasculitis (Churg-Strauss syndrome)   Saddle nose : trauma / polykondritt / Wegener’s granuolomatosis/ syfilis/ kokaine misbruk

16 Hand artrosis heberdense – bouchardse knuter

17 Hand chondrocalcinosis

18 A single 35 year-old woman with a history of Raynaud’s phenomenon since 1990 came to the Rheumatology outpatient clinic for evaluation. Her past medical and family history were unremarkable. Physical examination showed that the hands were cold with a bluish appearance and livedo reticularis was present on the lower extremities (figure). Immunological evaluation showed: positive antinuclear antibodies at low titer (1/80), positive anticardiolipin antibodies, both IgG and IgM (280 IU and 150 IU respectively, normal value <100 IU). The rest of the immunological evaluation was negative. What is the differential diagnosis? 1. Systemic lupus erythematosus   2. Progressive systemic sclerosis  Wrong answer 3. Primary antiphospholipid syndrome 4. Atrophic blanche   A number of conditions may cause the appearance of livedo reticularis: Cutis marmorata telangiectatica congenita a rare congenital condition Sneddon syndrome - association of Livedoid vasculitis and systemic vascular disorders, such as strokes, due to underlying genetic cause.[1] Idiopathic livedo reticularis - is the commonest cause of livedo reticularis appearance and is a completely benign condition of unknown cause affecting mostly young women during the winter.[2] May be mild, but ulceration may occur later in the summer.[3] Secondary livedo reticularis: Vasculitis autoimmune conditions: Livedoid vasculitis - with painful ulceration occurring in the lower legs Polyarteritis nodosa Systemic lupus erythematosus[4] Dermatomyositis Rheumatoid arthritis Lymphoma Pancreatitis[5] Tuberculosis Amantadine (drug) Obstruction of capillaries: Cryoglobulinaemia - proteins in the blood that clump together in cold conditions.[6] Antiphospholipid syndrome due to small blood clots Hypercalcaemia (raised blood calcium levels which may be deposited in the capillaries) Haematological disorders of Polycythaemia rubra vera or Thrombocytosis (excessive red cells or platelets) Infections (syphilis and tuberculosis) Arteriosclerosis (cholesterol emboli)[7][8] and homocystinuria (due to Chromosome 21 autosomal recessive Cystathionine beta synthase deficiency) Intra-arterial injection (especially in drug addicts Livedo reticularis

19 Primaer Raynaud prevalens 5-10% , ANA neg, Norm kapillaroskopi

20 DIP ledds artritt ved psoriasis (25% of cases)

21 Positiv ANA immunofluoresence technique
Fig ANA patterns on mouse liver. (a) Peripheral (rim) pattern; (b) homogeneous (diffuse) pattern; (c) speckled; (d) nucleolar. (Provided by Peter H Schur MD for this publication as well to UpToDate.) Positiv ANA immunofluoresence technique

22 Table 20.1 Sensitivity of the ANA in autoimmune and non-rheumatic disease

23 ANA ELISA assay

24 Fig Erythema nodosum – tender subcutaneous nodules on the lower legs. (Courtesy of St Mary's Hospital Medical School, London.) Erythema nodosum

25 Stage 3 sarcoidosis

26 Fig Erythema migrans. This annular, erythematous lesion developed over a period of 3 weeks around the site of a tick bite. (With permission from McKee.30) Erytema migrans

27 Cutan urinesyre ansamling = Tophi

28 Fig Acute gout. The first MTP joint is involved at some time in approximately 75% of patients. Desquamation of the skin often occurs.

29 Fig. 26. 7 Annular variety of subacute cutaneous lupus
Fig Annular variety of subacute cutaneous lupus. (With permission from Sontheimer et al.27) Subakutt kutan lupus

30 Hydrops/synovia-hyperplasi i kneledd (UL)
Fig Ultrasound monitoring of joint effusion. Images of the knee joint obtained in a patient with SLE arthritis demonstrate a large joint effusion (large arrows) and nodular areas of synovial hypertrophy (small arrows). (Courtesy of Dr Carol Benson, Brigham and Women's Hospital, Harvard Medical School.)

31 Rheumatoid arthritis with hyperplastic
synovial villi eroding and replacing cartilage at the joint margin

32 classic "bamboo" spine

33 Pølsefingre-tåer (dactylitt) ved PsA
Fig Sausage fingers and toes. (a) Psoriatic arthropathy – dactylitis at the third finger. (b) Reactive arthritis – dactylitis more evident at the second and third left toes and nail dystrophy. Pølsefingre-tåer (dactylitt) ved PsA

34 Fig Dermatomyositis. Dermatomyositt

35 Lupus Erythematosus - Malar eminences and nose - Malar erythema and atrophy.
Fig Acne rosacea.

36 Kapillæroskopi - scleroderma
Fig Nailfold capillary pattern in a patient with scleroderma. Notice the avascular areas. (Courtesy of Dr Hildegard Maricq.) Kapillæroskopi - scleroderma

37 Nailfold capillaroscopy in patients with systemic sclerosis showing changes from normal (top left): irregular vessels, loss of some, dilation of others

38 Fig cANCA pattern. Demonstration of cytoplasmic antineutrophil cytoplasmic antibodies (cANCAs) by indirect immunofluorescence with normal neutrophils. There is heavy staining in the cytoplasm while the multilobular nuclei (clear zones) are non-reactive. These antibodies are usually directed against proteinase 3 and most patients have Wegener's granulomatosis. (Courtesy of Dr Helmut Rennke.) Positive C-ANCA

39 Table 20.4 Significance of cANCA directed against proteinase 3

40 TA skalp nekrosis

41 Keratoderma blenorrhagicum (reaktiv artritt)
Fig Keratodermia blennorrhagicum on the soles of a patient with reactive arthritis. Keratoderma blenorrhagicum (reaktiv artritt)

42 Purpura (vaskulitt/ITP/fulminans)
Fig Palpable purpura of necrotizing venulitis. Purpura (vaskulitt/ITP/fulminans)

43 Fig Rheumatoid vasculitic ulceration in various stages of evolution. (With permission from Dieppe PA et al. Slide atlas of clinical rheumatology. London: Gower Medical Publishing 1983.) RA vaskulitt

44 Macroglossi kutane nedslag
AMYLOIDOSIS Macroglossi kutane nedslag

45 Diffuse Idiopathic Skeletal Hyperostosis,
( DISH /Forestier disease)

46 Fig. 27.3 Chronic uveitis in seronegative juvenile arthritis – 'white eye' uveitis.
Uveitt (JRA/SpA)

47 Fig Acute, severe, retinal vasculitis affecting the upper temporal retinal vessels. Note the large number of hemorrhages and white choroidal and retinal infiltrates. Retina vaskulitt

48 Neuropathic (Charcot) arthropathy

49 Libman Sacks endokarditt (SLE)
Fig Transesophageal echocardiogram in a patient with systemic lupus erythematosus. There is thickening of the anterior (aml) and posterior (pml) mitral leaflets and a Libman–Sacks vegetation (arrow) on the pml. (Courtesy of CA Roldan.) Libman Sacks endokarditt (SLE)

50 Aktivert human T-lymphocyte


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