AUTOIMMUNITY-I,II, III PRACTICAL 4 Case No 1 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. On physical examination she has mild leg edema. On auscultation, a friction rub is audible over the chest. Laboratory findings include pancytopenia and serum creatinine 3 mg/dL. Urinalysis shows hematuria and proteinuria. Serologic test for Auto-antibodies. 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 dermatitis with deposition of IgG in BM Sterile non-infective vegetations, called LibmanSacks endocarditis, are on BOTH sides of the leaflet. Questions Which mechanisms is most likely involved in the pathogenesis of her disease? How to establish the Diagnosis? Write the suitable laboratory diagnosis to reach the diagnosis? Mention the signs of severity in previous case? How to establish the Diagnosis History: characteristic symptoms Classical physical findings and signs I)Skin-Malar rashes(erythematous)+ Discoid rashes (scaly) 2) Arthritis, 3)photo-sensitivity. 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), Name of test Name of test Urine analysis skin biopsy & IF CBC+PBF Renal biopsy & IF ESR, CRP, LE-cells test RFT, LFT Imaging studies Auto-antibody type ANA Anti ds DNA ANTI-sm Anti-phospholipid Target components Nuclei Nuclear genome Nuclear protein Cell Phospholipid RF The lupus erythematosus (LE) cell test- is a diagnostic test of SLE LE cell is a neutrophil or macrophage that has phagocytized (engulfed) the denatured nuclear material of another cell. Case No -2 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. There is a subcutaneous nodule on the ulnar aspect of the right forearm. 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 Questions Which mechanisms is most likely involved in the pathogenesis of her disease? How to establish the Diagnosis? Write the suitable laboratory diagnosis to reach the diagnosis? How to establish the Diagnosis History: characteristic symptoms Classical physical findings and signs I) Articular symptoms& signs: symmetrical joints (> three), deformities, morning stiffness, Arthritis, 2) Muscular atrophy- bone erosion- articular cartilage surface damaged.- synovitisthickening- fibrosis- calcification. 3) Sub-cutaneous nodules 4) systemic – fever, fatigue, vascuiltis, heart, , etc.. Name of test Urine analysis CBC+PBF ESR, CRP, RFT, LFT+ Imaging study of joint and bone Auto-antibody type RF Anti-CCP (cyclic citrullinated peptide) HLA Non-HLA Case No 3 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. On physical examination, she has keratoconjunctivitis. Atrophy of the oral mucosa, with buccal mucosal ulcerations, also is present. A biopsy specimen of the lip shows marked lymphocytic and plasma cell infiltrates in minor salivary glands. Questions Which of the following antibodies is most likely to be identified on laboratory testing? ANA, RF, Anti SS-B (La), Anti SS A (R0) How to establish the Diagnosis? Write the suitable laboratory diagnosis to reach the diagnosis? Name of test Urine analysis CBC+PBF ESR, CRP, RFT, LFT+ Imaging study of joint and bone 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 Thanks