AUTOIMMUNITY-I,II, III PRACTICAL 4

l Case No 1 l A 25-year-old woman has had increasing malaise, a skin rash of her face exacerbated by sunlight exposure, arthralgias and myalgias for the past month. l On physical examination she has mild leg edema. On auscultation, a friction rub is audible over the chest. l Laboratory findings include l pancytopenia and serum creatinine 3 mg/dL. l Urinalysis shows hematuria and proteinuria. l Serologic test for Auto-antibodies. l A renal biopsy shows granular deposits of IgG and complement in the mesangium &BM.

Malar rash butter-fly (rash on cheeks )

Discoid rash

Lupus nephritis : a glomerulus with several "wire loop" lesions representing extensive subendothelial deposits of immune complexes

SLE- skin lesion show vacuolar interface dermatitisSLE- skin lesion show vacuolar interface dermatitis with deposition of IgG in BM

Sterile non-infective vegetations, called Libman- Sacks endocarditis, are on BOTH sides of the leaflet.

l Questions l Which mechanisms is most likely involved in the pathogenesis of her disease? l How to establish the Diagnosis? l Write the suitable laboratory diagnosis to reach the diagnosis? l Mention the signs of severity in previous case?

How to establish the Diagnosis l History: characteristic symptoms l Classical physical findings and signs l I)Skin-Malar rashes(erythematous)+ Discoid rashes (scaly) l 2) Arthritis, l 3)photo-sensitivity. l SLE is summarized in “SOAP BRAIN MD” Four out of eleven of : (S=serositis, O=oral ulcers, A=arthritis, P=photosensitivity, pulmonary fibrosis, B=blood cells, R=renal, Raynauds, A=ANA, I=immunologic (anti-Sm, anti-dsDNA), N=neuropsych, M=malar rash, D=discoid rash), l

Target componentsAuto-antibody type RF Nuclei ANA Nuclear genome Anti ds DNA Nuclear protein ANTI-sm Cell Phospholipid Anti-phospholipid Name of test skin biopsy & IFUrine analysis Renal biopsy & IFCBC+PBF LE-cells testESR, CRP, Imaging studiesRFT, LFT

The lupus erythematosus (LE) cell tes t- is a diagnostic test of SLE LE cell is a neutrophil or macrophage that has phagocytized (engulfed) the denatured nuclear material of another cell.neutrophilmacrophage

l Case No -2 l A 57-year-old woman has had increasing pain and deformities in her hands for the past 10 years. On physical examination, she has bilateral ulnar deviation and swan-neck deformities of several fingers. l There is a subcutaneous nodule on the ulnar aspect of the right forearm. l A biopsy specimen of the nodule sent for histopathology?

Sub-cutaneous nodule

RA- narrowing of joint space and marginal erosions on both radial& Ulnar + dislocation

RA: synovial hypertrophy with formation of villi, subsynovial tissue containing a dense lymphoid aggregate

Pannus=Subcutaneous rheumatoid nodule with an area of fibrinoid necrosis (top) surrounded by a palisade of macrophages& scattered chronic inflammatory cells

l Questions l Which mechanisms is most likely involved in the pathogenesis of her disease? l How to establish the Diagnosis? l Write the suitable laboratory diagnosis to reach the diagnosis?

How to establish the Diagnosis l History: characteristic symptoms l Classical physical findings and signs l I) Articular symptoms& signs: symmetrical joints (> three), deformities, morning stiffness, Arthritis, l 2) Muscular atrophy- bone erosion- articular cartilage surface damaged.- synovitis- thickening- fibrosis- calcification. l 3) Sub-cutaneous nodules l 4) systemic – fever, fatigue, vascuiltis, heart,, etc..

Auto-antibody type HLA RF Non-HLAAnti-CCP (cyclic citrullinated peptide) ANA Name of test Urine analysis CBC+PBF ESR, CRP, RFT, LFT+ Imaging study of joint and bone

l Case No 3 l A 45-year-old woman has had a burning sensation with increasing blurring of vision for the past 5 years as well as persistent difficulty in swallowing. l On physical examination, she has keratoconjunctivitis. Atrophy of the oral mucosa, with buccal mucosal ulcerations, also is present. l A biopsy specimen of the lip shows marked lymphocytic and plasma cell infiltrates in minor salivary glands.

l Questions l Which of the following antibodies is most likely to be identified on laboratory testing? l ANA, RF, Anti SS-B (La), Anti SS A (R0) l How to establish the Diagnosis? l Write the suitable laboratory diagnosis to reach the diagnosis?

Auto-antibody type Anti SSa\R0= target nucleic acids in cytoplasm. Anti-SSb (La)=protein b\w nuclei&cytoplasm ANA= nucleic acid in nuclei+ HLA- DR3, etc Name of test Urine analysis CBC+PBF ESR, CRP, RFT, LFT+ Imaging study of joint and bone

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