AUTOIMMUNE Dz ANTI – RO =SJOGRENS, RA, SLE ANTI -SSA -SSB =SJOGRENS, RA, SLE FANA/ANA =SLE ANTI -SM =SLE ANTI – JO-1 =POLYMYOSITIS ANTI -CENTROMERE =CREST VARIANT OF PSS (SCLERODERMA) ANTI -SCL -70 (DNA TOPOISOMERASE I) =SCLERODERMA MS—HIGH MYELIN BASIC PROTEIN (IgG) MG—IgG TO POST SYNAPSE PVS—? POLYMYOSITIS-DERMYOSITIS—-ANTI-JO-1 RELAPSING POLYCHONDRITIS—ANTI TYPE III COLLAGEN ANTIBODIES PEMPHIGUS–IgG AGAINST AGAINST SQUAMOUS EPITHIAL CELL SURFACES HASHIMOTOS THYROIDITIS—-ANTIMICROSOMAL/PEROXIDASE IgG, ANTI-THYROGLOBULIN IgG, ANA GRAVES— IgG– LATS 68 KD PROTEIN=75% CORRELATION WITH AUTOIMMUNE INNER EAR Dz C-ANCA (POSSIBLY P-ANCA)=WEGENER’S GRANULOMATOSIS VASCULIDITIES—-A SUBSET OF THE AUTOIMMUNITIES BEHCET’S Dz (SYNDROME) BEHCET = A TURKISH DERMATOLOGIST (1937) A CHRONIC IDIOPATHIC SYSTEMIC VASCULITIS (INFLAMMATORY DISORDER) MALE > FEMALE 3RD DECADE HIGH INCIDENCE IN TURKEY, JAPAN, EUROPE MAJOR TRIAD: RECURRENT APTHOUS ULCERS (100%), GENITAL ULCERS(65%), OCULAR INFLAMATION (IRIDOCYCLITIS)—-CAN LEAD TO BLINDNESS IN ABOUT 3 YEARS!!!!—ALSO PROMINENT IS CUTANEOUS VASCULITIS(HYPERIRRITABLE SKIN) APTHOUS ULCERS ARE CHARACTERISTICALLY “PUNCHED OUT” WITH SURROUNDING ERYTHEMA AND COVERED WITH A PALE PSEUDOMEMBRANE—-PAINFUL–OCCUR IN CLUSTERS PROGRESSIVE SNHL AN AVERAGE OF 4 YEARS FROM ONSET TO DIAGNOSIS OFTEN + FHx MINOR Sx: ARTHRALGIAS, MENINGOENCEPHALITIS(LATE=ASEPTIC MENINGITIS—-THE PRIMARY CAUSE OF DEATH), FORCED LAUGHTER, THROMBOPHLEBITIS(DVT), BULBAR PAULSEY, ESOPHAGITIS, COLITIS, C-V DISORDER, GLOMERULAR NEPHRITIS LEUKOCYTOSIS HIGH ESR AND CIRCULATING IMMUNE COMPLEXES, HIGH AB TO MUCOSAL CELLS EOSINOPHILIA Rx: COLCHICINE FOR ULCERS, STEROIDS, AZOTHIAPRINE, CYCLOSPORIN A POSSIBLE SURGERY CHURG-STRAUSS SYNDROME (EOSINOHILIC ASTHMA) ALLERGIC ANGIITIS–A MULTISYSTEM GRANULOMATION VASCULITIS LIKE A COMBINATION OF RELAPSING POLYCHONDRITIS AND ASTHMA CAN BE UNMASKED BY NEW ANTI-LEUKOTRIENE ASTHMA MEDS 50% 5 YEAR SURVIVAL FEMALE 1.3:1 MALE 3 STAGES: PRODROMAL: RHINITIS, NAO, BRONCHIAL ASTHMA (LUNGS ARE ALWAYS INVOLVED) HYPEREOSINOPHILIC PHASE WIDE SPREAD (SYSTEMIC) VASCULITIS ANCA – (A CLINICAL Dx) Rx: STEROIDS COGAN’S SYNDROME “Non-Syphilitic Interstitial Keratitis” A Systemic Vasculitis (Probably Autoimmune in Etiology)--HYDROPIC SYMPTOMS WITH SPIRAL GANGLIONITIS Primarily Affects Young Adults FEMALE >MALE 1. Severe Episodic Vertigo/Nystagmus/ Ataxia/Unsteadiness 2. Progressive SNHL—MAY BE REVERSED WITH STEROIDS 3. Tinnitus 4. AURAL FULLNESS Peripheral (Vs. central found in syphilis–USUALLY OLDER PTS) Fiery Episceritis “Corneitis/keratitis” OTHER DIFF Dx = VOGT-KOYANIGI-HARADA SYNDROME (ALOPECIA AND EXUDATIVE UVEITIS) & SYPHILIS 1-2 WEEKS Malaise/Arthralgias SKIN NODULES Aortitis–AI GI TRACT INVOLVEMENT High ESR Eosinophilia Rx:
Ophthalmologic Consultation Cyclophosphamide Azathioprine Prednisone Steroid Eye Drops POLYARTERITIS NODOSA A VASCULITIS OF MID AND SMALL SIZED ARTERIES SNHL, FACIAL PAULSEY, ARTHRITIS, HTN, MYOPATHY,SKIN LESIONS HIGH ESR, + P-ANCA TEMPORAL GIANT CELL ARTERITIS—CRANIAL ARTERITIS, GRANULOMATOUS ARTERITIS A H&N VASCULITIS 50% COMORBID WITH PMR (POLYMYALGIA RHEUMATICA)—-PMR IS 2-3X MORE COMMON—-MUSCULOSKELETAL PAIN IN NECK, SHOULDERS, PELVIS ECT. ANEMIA, DEPRESSION, WT LOSS, AM STIFFNESS, FATIGUE, LOW GRADE FEVER, MALAISE FOR GREATER THAN 1 MONTH TEMPORAL ARTERITIS = A COMMON IDIOPATHIC SMOALL VESSEL VASCULITIS—-RESULTANT ISCHEMIA FEMALE > MALE 90% > 60 90% UNILATERAL?/BILATERAL? HA–INTENSE PULSATILE CEPHALALGIA, HYPERALGESIA OF THE SCALP, JAWS, TONGUE, AND NECK—MASTICATORY CLAUDICATION, ODYNOPHONIA, ANOREXIA, MALAISE, FEVER, ½ OF THE TIME WILL HAVE STREAKING ERYTHEMA OVER THE TEMPPORAL ARTERY ESR > 40—OFTEN >100 LOW ALBUMIN AND HIGH IMMUNOGLOBULIN COUNTS 1/3 PTS HAVE OPHTHO INVOLVEMENT BLINDNESS IN 1/3 OF UNTREATED PTS—OFTEN HERALDED BY AMAUROSIS FUGAX IF SUSPICIOUS—START STEROIDS—60MG IV NEED TO DO TEMPORAL ARTERY BIOPSY WITHIN 72 HOURS OF STARTING STEROIDS—TAKE 6 CM SEGMENT USUALLY OF THE POST BRANCH (SKIP LESIONS–A 30% INCIDENCE)—-DO NOT INJECT WITH EPI OR YOU MAY LOSE PULSATIONS TO GUIDE THE DISSECTION—-CAN USE DOPPLER IF YOU WANT IF BIOPSY NEGATIVE GO TO THE OTHER SIDE MAY NEED TO TREAT WITH STEROIDS UP TO 2 YEARS——-NEVER MISDIAGNOSE AS MIGRAINE AND NEVER TREAT WITH ERGOTAMINES—-CAN QUICKLY GO BLIND WEGENER’S GRANULOMATOSIS DESCRIBED IN 1936 BY FREIDRICH WEGENER AN ASEPTIC (AUTOIMMUNE) IDIOPATHIC NECROTIZING GRANULOMATOUS VASCULITIS(BOTH A VASCULITIS AND A GRANULOMATOUS Dz) FEMALE 3:2 MALE ? CHILDREN CAN GET IT USUALLY AGE 30-40 SYMPTOM TRIAD OF: 1. UPPER RESP TRACT NECROTIZING GRANULOMAS NOSE: FIRST SYMPTOM = SINUSITIS RHINITIS, EPISTAXIS, ULCERATIONS, POST. BONEY (VOMER) SEPTAL PERF–SADDLE NOSE—DO NOT OPERATED ON THEM! LARYNX–SUBGLOTTIC STENOSIS (STRIDOR)—HIGHER INCIDENC IN PATIENTS < 20—?FREE FLAP LTP EAR– #1 ETD–OME (CHL), #2 MASTOIDITIS (CAN EVEN GIVE FACIAL PARESIS)—MOST OFTEN MISTAKEN FOR AURAL TB (HAVE CAVITARY PULM LESIONS WITH SIMILAR INFLAMMATORY FINDINGS), #3 SSNHL 2. DISSEMINATED VASCULITIS OFTEN AFFECTS THE LUNG–CAVITARY LUNG LESIONS—HEMOPTYSIS 3. CRESCENTRIC FOCAL GLOMERULAR NEPHRITIS–CLASSIC RBC CASTS-HEMATURIA, PROTEINURIA(HIGH UA SED RATE) RENAL STATUS = THE MOST IMPORTANT PROGNOSTIC INDICATOR ALSO CONSTITUTIONAL Sx: FEVER, MALAISE Dx: Hx, UA, HIGH ESR, ANEMIA, THROMBOCYTOPENIA, CXR-CAVITARY PULM NODULES, HIGH Cr, C-REACTIVE PROTEIN + C-ANCA (IgG AUTO Ab—ANTI NEUTROPHILIC CYTOPLASMIC ANTIBODIES)–92-93%, + ANTI-PROTEASE – 3 P-ANCA MAY ALSO BE IMPORTANT BIOPSY: LUNG = BEST, THEN KIDNEY—NASAL Bx = GET 10 SPECIMENS SILVER METHAMINE STAIN–PLASMA CELLS MULTINUCLEATED LANGHANS GIANT CELLS, HISTIOCYTES, ISCHEMIC GEOGRAPHIC NECROSIS–BASOPHILIC “SMUDGY” NECROSIS Rx: COMBO TREATMENT IS BEST STEROIDS 1MG/KG/DAY,PULSED CYTOXAN IF RENAL INVOLVEMENT (CYCLOPHOSPHAMIDE)–(HEMORRAGIC CYSTITIS, BLADDER CA, MYELODYSPLASIA), IMMURAN (AZATHIOPRINE), TMP/SMX—-VERY SUSPECT, MTX, NS IRRIGATIONS (SUPERINFECTION OFTEN IS S.a) REMISSION RATES 70-85% AVOID RT