DisorderVasculitisEpidemiology/EtiologyClinical/Laboratory FindingsTreatment

Takayasu arteritispulseless disease Large Vessel Granulomatous Aortic Arch vesselsYoung Asian women and children No Upper extremity pulse >10 mmHg discrepancy in BP between arms Visual Defects StrokeCorticosteroids

Giant Cell (Temporal) Arteritis Large Vessel Granulomatous Involving superficial temporal and ophthalmic arteriesAdults > 50 Temporal headache Jaw claudication I/L blindness due to ophthalmic artery vasculitis Polymyalgia rheumatica is commonly associated (NL CK) ESR = useful screeningCorticosteriods

Polyarteritis Nodosa (PAN) Medium-sized vessels Necrotizing Involving renal, coronary, mesenteric arteries Pulmonary arteries SPARED Middle-aged men HBsAg association (immunocomplex disease; Type III HSV) HCV less common UNK cause if not one of above Vessels are at ALL stages of Acute and Chronic inflammation Fever (commonly present; unk origin) Aneurysms (produced by focal vasculitis; detected w/angiography) Organ infarction in Kidneys (renal failure, hematuria), Heart (acute MI), Bowels (bloody diarrhea), Skin (ischemic ulcer), Testicle (testicular pain) Corticosteroids Cyclophosphamide in resistant cases

Kawasaki Disease Medium-sized vessel Necrotizing Coronary arteries Children < 5 yo Boys > girls UNK cause (likely infectious cause in genetically susceptible ppl) Leading cause of acquired hear disease in children in developed countries Asian children = Highest incidence (Japan) Fever Erythema and edema of hands and feet - convalescing w/desquamated rash cervical adenopathy oral erythema & cracking of lips Glossitis (strawberry-appearing tongue) AbNL ECG IV Ig Aspirin Corticosteroids (only after 2 unsuccessful courses of IV Ig)

Thromboangitis Obliterans(Buerger Disease) Medium-sized vessels digital vessel thrombosis & damage to neurovascular compartment Men 25-50 yo Smokers Genetic mechanism MC - Ashkenazi Jews in Israel High morbidity in India, Korea, and Japan 10% in females Lower extremity involvement (100% cases) resting pain on forefoot possible ischemic ulcers or gangrene of foot/toes Upper extremity involvement (40-50% cases) upper limb ischemia w/ulceration and gangrene (amputation common) Raynaud phenomenon Smoking cessation essential IV iloprost (PG analogue) Vasodilators (-blockers, Ca-channel blockers)

Raynaud Disease Medium-sized vessels Digital vessels in fingers and toes tip of nose and ears in some cases Young women Exaggerated vasomotor response to cold or stress Paroxysmal digital color changes (white-blue-red sequences) Ulceration and gangrene in chronic cases Avoid cold temperatures (wear gloves) Ca-Channel blockers (nifedipine)

Raynaud Phenomenon Medium-sized vessels digital vessels in fingers and toes Tip of nose and ears in some cases Adults (men and women) Secondary to other diseases (SLE, CREST, Systemic sclerosis) Systemic Sclerosis and CREST digital vasculitis w/vessel fibrosis dystrophic calcification ulceration gangreneSame as for Raynaud Disease Avoid cold temperatures (wear gloves) Ca-Channel blockers (nifedipine)

Wegener Granulomatosis Medium and Small-Sized vessels Necrotizing involving Lung (infarctions) and Renal Vessels (glomerulonephritis) 41 yo = mean Incidence equal among genders ~ 90% present with Upper/Lower airway symptoms Necrotizing Granulomas in Skin URT (nasopharynx-saddle nose deformity, chronic sinusitis, trachea collapse) LRT (cavitating nodular lesions) Necrotizing vasculitis in lungs (infarction, hemoptysis) Kidneys (crescentic glomerulonephritis) c-ANCA antibodies (>90%) correlate erratically w/Tx Corticosteroids Cyclophophamide

Microscopic Polyangitis Small vessel skin lung brain GI tract Kidneys (postcapillary venules and glomerular capillaries) Children and adults Precipitated by drugs, infections, and immune disorders (penicillin, streptococci, SLE) vessels at SAME stage of inflammation Palpable purpura glomerulonephritis (crescentic) p-ANCA Abs (>80%)Corticosteroids and cyclophosphamide

Churg-Strauss Syndrome Small vessel skin lung heart vessels 51 yo = mean likely autoimmune Allergic rhinitis asthma p-ANCA (70%) eosinophilia corticosteroids

Henoch-Schonlein Purpura Small vessel skin GI Renal Joint vessels Children 1-15 yo some young adults Peak incidence in Spring (rarely summer) Males > females MC vasculitis in children MC Whites & Asians than blacks IgAAnti-IgA immunocomplexes (Type III HSR) Often follows: Viral URI GAS pharyngeal infection Pathogens may act as Ag trigger that causes Ab formation leading to IC formation (Type III HSR) Palpable purpura: bottocks and lower extremities (95-100%) Polyarthritis (80%) Glomerulonephritis (80%) Abd pain and vomiting (85%) GI bleeding Recurrence in 1/3 of cases Most = spontaneous recovery in 4 mo w/o Tx Corticosteroids (if severe GI disease or renal disease are present)

Cryglobulinemia Small vessel skin GI Renal Different types of cryglobulinemia (mixed, monoclonal, polyclonal) Adults female > male (3:1) Associations with HCV (>50%) Type 1 MPGN Multiple Myeloma (monoclonal type) Lymphoproliferative disorders CT disorders Cryglobulins: proteins in plasma gel at cold temperatures particularly in those areas exposed to cold temperatures Palpable purpura Acral cyanosis of nose and ears Raynaud phenomenon Glomerulonephritis (crescentic) Arthritis Abd painImmunosuppressive agents

Infectious Vasculitis Small vessel Skin vessels Children and adults involves Microbial Pathogens Rocky Mountain Spotted Fever (RMSF) MC in SE, followed by south central states RMSF: Dog tick or wood tick transmission of Rickettsia rickettsii (Dermacentor variables or andersoni) Organisms invade endothelial cells producing vasculitis Fever (100%) Petechiae begin on palms spread to trunk 50% appear 1st day 80% appear by 5th day 10% no petechiae Oral Doxycycline Can treat children up to 10 days