Medium Vessel Vasculitides

Snapshot
A patient presents with a fever, diffuse myalgias, abdominal pain, a peripheral neuropathy, as well as hypertension and a recent weight loss of 10 pounds.  The patient has a past medical history of hepatitis B and C.  Angiography demonstrates a “string of pearls appearance” in the renal artery, as well as in several other organ systems with the exception of the pulmonary arteries.
Polyarteritis Nodosa
Introductionimmune complex-mediated transmural vasculitis with fibrinoid necrosisinvolves renal, coronary, and mesenteric arteriesdoes not involve the pulmonary arterieslesions of different ages (the rationale being that circulating immune complexes deposit at different times)Symptomsflu-like symptomsfever, malaise, and myalgiaGI symptomsweight loss, abdominal pain, melenahypertensionneurologic dysfunctioncutaneous eruptionsFindingshepatitis B (HBsAg) seropositivity in 30% of patients multiple aneurysms and constrictions on arteriogram segmental ischemic necrosis with lesions of different ages increased inflammatory markers like ESR and CRPTreatmentcorticosteroidscyclophosphamide
Snapshot
A 7-year-old male presents with conjunctival injection and a rash that has persisted for quite some time.  On physical exam, the patient has a bright red tongue, adenopathy, and hepatosplenomegaly as well as a fever that has persisted for the past 7 days.
Kawasaki Disease (Mucocutaneous Lymph Node Syndrome)
Introduction acute, self-limiting necrotizing vasculitis coronary arteries often affected (thrombosis and aneurysm)seen in infants and childrenleading cause of death from acquired heart disease in childrenassociation with Asian ethnicityPresentation feverconjunctivitiscervical lymphadenitisdesquamative skin rashwith changes in lips/oral mucosa (“strawberry tongue”)myocardial infarction (if coronary arteries affected)crash and burnconjunctivitisrashadenopathystrawberry tonguehands (peeling skin)feverTreatment IV immunoglobulinaspirinvaccinations (do not want the patient to get a viral infection due to aspirin treatment)
Snapshot
A 69-year-old gentleman presents with painful discoloration and ulceration of his fingers. The patient’s symptoms have been slowly worsening over the past several months.  This patient has smoked 3 packs of cigarettes per day for the past 40 years.
Thromboangiitis Obliterans (Buerger Disease)
Introductionvasculitis with digital vessel thrombosisseen in heavy smokers, most commonly in men between ages 25 and 50 thought to be caused by a hypersensitivity reaction to components of tobacco smokePresentationintermittent claudicationpainful ulceration due to the involvement of nerve and vesselRaynaud’s phenomenonmay lead to gangrene and autoamputation of digitsTreatmentsmoking cessationcilostazoliloprostcalcium channel blockers (nifedipine)
Snapshot
A 49-year-old woman with abnormally smooth skin on her face and hands presents with a chief concern of episodes of intense pain in her hands.  She states that her hands at times will turn pale, then will flush red after a short period of time.  She describes the episodes as very painful and unpleasant.
Raynaud’s Disease
Introductionvasculitis affecting vessels of fingers and toes ↑↑ vasomotor reaction to cold/stressseen in young womenPresentationdigital color changes (white to blue to red)chronic cases may progress to ulceration and gangreneDiagnosisnail fold capillaroscopy Treatmentcalcium channel blockers (nifedipine, amlodipine) sildenafil (phosphodiesterase inhibitor)
Raynaud’s Phenomenon
Introductionvasculitis affecting vessels of fingers and toesthe phenomenon as result of an underlying disease   e.g., CREST syndrome and systemic sclerosisaffects adults (no gender specificity)

Presentationdigital color changeschronic cases may progress to ulceration and gangreneTreatment

lifestyle and general

avoid triggers such as cold and stresscessation of smokingavoid medications that can precipitate attacks such as beta-blockers and ergotaminesmedicalcalcium channel blockers (nifedipine)

first-linetopical nitroglycerinsecond-line