Systemic Lupus Erthematosus SLE

-Is a asystemic disease in which tissue and multiple organs are damaged by pathogenic autoantibodies and immune complexes (it is a type 3 hypersensitivity reaction caused by antibody-immune complesx formation) (PIP & MCP)

-SLE most often harms (heart,joints,skin,lungs,blood vessels,liver,kidneys and nervous system)

-The disease occurs nine times more often in women than in men (women in childbearing yrs ages 15-35yrs)

-Symptoms & Signs :

*Dermatological maifestations

.malar rash (or butterfly rash)

.red scaly patches on the skin

.alopacia

.mouth,nasal,urinary tract and vaginal ulcers

*Musculoskeletal

.the small joint of the hand and wrist,although all joints are at risk

+Proximal intraph. Joint (don`t affect)

.SLE is less disabling and usually does not cause severe destruction of the joints (unlike RA)

*Hematological

.anemia,low platelet and WBC

.antiphospholipid antibody syndrome (high PTT/reccurent abortion/reccurent DVT)

.anticardiolipin antibody (positive test for syphilis)

*Cardiac

.pericarditis

.myocarditis

.endocarditis

Noninfective (libman-sacks endocarditis)

*Pulmonary

.pleuritis

.pleural effusion

*Renal

.hematuria

.proteinuria

.acute or end stage renal failure

*Neurological

.headaches depression

.seizures

.cognitive dysfunction

.mood diorder

.cerebrovascular disease

.polyneuropathy

.anxiety diorder

.psychosis

*Reproductive

.increased rate of fetal death in utero and spontaneous abortion

*Other

.fever

.malaise

.joint pains (myopathy)

.myalgias

.fatigue

.abdominal pain

-Causes:

.genetics environmental triggers (drug reactions-reversible)

(M.C of which are : procainamide,isoniazid,hydralazine,quinidine,phenytoin)

-Diagnosis :

.antinuclear antibody (ANA) testing

.antiextractable nuclear antigen (anti-ENA) (serologic form testing for SLE)

-Criteria :

.person has SLE if any 4 out of 11 symptoms are present

.malar rash (rash on cheeks)

.discoid rash (red,sdaly patches on skin that cause scarring)

.serositis (pleurisy-inflammation of the membrane around the lungs)

.pericarditis (inflammation of the membrane around the heart)

.oral ulcers

.arthritis (tow or more peripheral joints,with tenderness,swelling or effusion)

.photosensitivity

.blood / hematological disorder (hemolytic anemia,leukopenia,lymphopenia,thrombocytopenia,neutropenia)

.hypocomplementemia

.renal diorder

.ANA test positive

.positive anti-smith,anti-ds DNA,antiphospholipid antibody,positive serological test for syphilis

.neurological disorder (seizures or psychosis)

-Treatment :

.includ corticosteroids and anti-malarial drugs (cyclophophamide ,mycophenolate and hydroxychloroquine)

.renal transplantation (for end-stage renal disease)

.neonatal lupus (most serious complication for neonatal lupus is heart block)

+SLE considered incurable,but highly treatable

**Gout (podagra)

-Is amedical condition usually characterized by recurrent attacks of acute inflammatory arthritis (red,tender,hot,swollen joints)

-The metatarsophalangeal joint MTP at rhe base of the big toe is the most commonly affected,other joints such as the heels,knees,wrists and fingers / It may also present as tophi,kidney stones or urate nephropathy

-Acute attacks of gout rarely affect the shoulder

-Causes :

.elevated levels of uric acid in the blood (hyper-uricemia)

-Symptoms & Signs :

.recurrent attack of acute inflammatory arthritis

.pain usually begins over 2-4 hours and during the night

.elevated levels of uric acid may also lead to crystals precipitating in the kidneys,resulting in stone formation (uric acid stones)

.include a strong association with the consumption of (alcohol,fructose-sweetened drinks,meat,seafood,physical trauma,surgery,coffe,vit C,dairy products)

.metabolic syndrome (abdominal obesity,hypertension,insulin resistance,abnormal lipid levels)

.diuretics have been associated with attacks of gout

.other medicines (niacin,aspirin ‘acetylsalicylic acid’)

.the immunosuppressive drugs (ciclosporin,tacrolimus)

-Common Gout Triggers :

.regular,excessive alcohol intake (beer)

.surgery or a sudden,severe illness

.consuming large quantitis red meat and shellfish

.a diet high in froctose

.radiation therapy

.crash diets,especially high-protein tad diets

.starting a uric acid-lowering treatment medicine

.taking the anti-rejection transplant medication cyclosporine (aspirin,duritics)

-Diagnosis :

.monosodium urate crystals in synovial fluid or a tophus (the most specific diagnostic finding ‘sodium urate crystals in synovial fluid’)

.needle-like morphology

.negative birefringencemia

-Treatment :

.NSAIDs (usual 1^st-line treatment for gout)

.colchicine (alternative for those unable to tolerate NSAIDs)

.glucocorticoids

-Prophylaxis :

.xanthine oxidase inhibitor (allopurinol / allopurinol not treatment) and uricosurics (probenecid)

**Pseudogout (CPPD)

-M.C in knee;other joitns commonly affected are the (wrist,shoulder and ankle) / is more common in elderly population and in people who have preexisting joint damage / Is caused by Ca urate

-Metabolic Abnormalities that are associated with CPPD :

.hyperparathyroidism

.hemochromatosis

.hypophosphatemia

.hypomagnesemia

-Diagnosis :

.rectangular crystal

.rhomboid crystal

.positive birfrin gent crystal on synovial fluid evaluation

.radiographs

-Treatment :

.is the same as gout

Scleroderma SSc

-Is a chronic systemic autoimmune disease (primarily of the skin-skin thickening)

-Disease characterized clinically by thickening of the skin caused by accumulation of connective tissue and by involvement of visceral organs (GI,lungs,kidneys / example ‘scleroderma renal crisis’-has been used to characterize the renal involvement in scleroderma)

-F > M (to develop scleroderma)

-Types :

1.limited Systemic Sclerosis

.scleroderma involves cutaneous manifestation that mainly affect the (hands,arms and face) and called CREST syndrome

.Pt with limited scleroderma ,the ANA test is positive

.Scle-70 test is negative

+CREST syndrome or Limited scleroderma :

.Calcinosis (calcium deposits occur in soft tissue usually in the fingers)

.Raynaud (raynaud phenomenon)

.Esophageal dysfunction

.Sclerodactery

.Telangiectasias

+Raynaud's phenomenon (Raynaud Disease) is seen in :(vasospastic diorder causing dicoloration of the fingers,toes)

.SLE

.Scleroderma

.Rheumatoid arthritis

.CREST

.Thoracic outlet syndrome

.Dermatomyosis

.Polymyositis

.Mixed connective tissue disease

.Ehlers-Danios syndrome

.Cold agglutinin disease

.Systemic sclerosis

.Cervical rip

+Primary Raynaud Disease (denotes a Pt without an associated underlying disease / It often develops in young women in their teens and early adulthood)

+Secondary Raynaud Disease (to describe Pt with a defined secondary or associated disease (scleroderma)

+One test that allows the differentiation between primary & secondary Raynaud is (Nailfold capillaroscopy test)

+Treatment for Raynaud Disease is (calcium-channel blockers as nifedipine)

2.Diffuse Systemic Sclerosis

.scleroderma is rapidly progressing and affects a large area of the skin and one or more internal organs (kidneys,esophagus,heart and lungs)

-Complication from Scleroderma include :

.heart (high Bp,irregular heart rhythm,enlargement of the heart lead to heart failure)

.kidney (scleroderma renal crisis,malignant hypertension,acute renal failure)

.lung (shortness of breath,coughing,difficulty breathing,a;veolitis,pneumonia,cancer,fibrosis)

.digestive (esophagus damage,acid reflux,malabsoption,dysphagia)

.skin & joints (carpal tunnel syndrome,muscle weakness,joint pain,stiffness)

-Treatment :

D-penicillamine (for the skin manifestation)

+In a patient with anaphylactic shock, all are correct treatments :

.Epinephrine

.Adrenaline

.IV fluids

.Steroids

+IgE mediated hypersensitivity reaction like bronchial asthma is (Type I hypersensitivity reaction)

Date: 2016-04-22; view: 551