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)–HYDROPICSYMPTOMS 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– #1ETD–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