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The term vasculitis encompasses a group of mostly uncommon diseases, all of which are characterized by blood-vessel inflammation, leading to a wide variety of clinical presentations.[1-6] Although these diseases may present with a multiplicity of findings, they can be distinguished on the basis of usual age of onset, specific pathologic features, and typical organ system involvement. The slide shows tender erythematous nodules with central "punched-out" ulcerations (arrows), which are common in cutaneous polyarteritis nodosa (PAN).
Image courtesy of Medscape.
Although overlap may be seen, the different forms of vasculitis may be distinguished by the size of vessels involved, ranging from vasculitides affecting the aorta and its main branches to vasculitides affecting the capillaries and venules. The clinical manifestations of vasculitis depend on the organ systems involved. Constitutional symptoms are common in many of the vasculitides.
Image courtesy of Medscape.
EGPA—eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome); GPA—granulomatosis with polyangiitis (Wegener granulomatosis); HSP—Henoch-Schönlein purpura; MPA—microscopic polyangiitis.
The workup of vasculitis begins with laboratory studies that include general tests (eg, complete blood count [CBC] with differential, erythrocyte sedimentation rate [ESR], and C-reactive protein [CRP]), organ-specific tests (eg, blood urea nitrogen [BUN], creatinine, liver function tests [LFTs], urinalysis, and lumbar puncture [LP]), tests for autoantibodies (eg, antineutrophil cytoplasmic antibody [ANCA]), and studies aimed at secondary causes as clinically indicated. Secondary causes may include infectious diseases (particularly beta-hemolytic streptococcal upper respiratory infection [URI] and tuberculosis [TB]), rheumatologic disease, malignancy, and medications. Drugs commonly linked to vasculitis include antibiotics, nonsteroidal anti-inflammatory drugs (NSAIDs), and diuretics; however, almost all drugs are potential causes.
The following cases present examples of vasculitis affecting large, medium-sized, and small vessels, along with examples of clinical features that may manifest in disease of various vessel sizes.[7]
Data in table from Jennette JC et al.[7]
A 38-year-old white man presents with a history of sinusitis unresponsive to treatment. He has recently developed a deformity of the bridge of the nose (shown). He complains of anorexia, low-grade fever and sweats, generalized arthralgia, and a productive cough that is blood-tinged. Urinalysis shows red blood cells (RBCs) and RBC casts. A chest radiograph is obtained, and autoantibody studies are ordered.
Which of the following is most likely to be apparent on the chest film?
Image courtesy of Medscape.
Nodular infiltrates are more easily appreciated on computed tomography (CT) or magnetic resonance imaging (MRI), as illustrated.
Which of the following is the most likely autoantibody finding in this patient?
Image courtesy of Medscape.
Answer: B. Positive cytoplasmic ANCA (C-ANCA).
Immunofluorescent staining of neutrophils from this patient's serum is shown above. C-ANCA staining consistent with anti-proteinase 3 antibodies is seen. A biopsy is obtained.
On the basis of the findings in this case, which of the following is the most likely diagnosis?
Image courtesy of Medscape.
Answer: B. Granulomatosis with polyangiitis (GPA; also termed Wegener granulomatosis).
As expected, the biopsy reveals a granulomatous vasculitis consistent with GPA (shown). GPA is characterized by sinusitis unresponsive to treatment, which often leads to collapse of the bridge of the nose ("saddle-nose" deformity), nodular pulmonary infiltrates, systemic symptoms, and glomerulonephritis.[8,9] C-ANCA is positive in approximately seven of eight patients and may be useful in monitoring disease activity.
A renal biopsy is also obtained. Which of the following is the renal biopsy likely to show?
Image courtesy of Medscape.
Answer: C. Focal glomerulonephritis.
A focal pattern of neutrophilic infiltrate (arrow) can be seen on this biopsy from a patient with GPA, consistent with a focal glomerulonephritis. Membranous disease and minimal-change disease are not features of GPA.[9] Renal arteritis is more typical of PAN. In addition to hematuria, renal manifestations of vasculitis include proteinuria and hypertension.
Image courtesy of Medscape.
A 68-year-old white man presents to the emergency department (ED) with abdominal pain, nausea and vomiting, and diarrhea. For the past 2 weeks, the patient has had a nodular erythematous rash (shown), arthralgia, and a low-grade fever. The ESR is 62 mm/hr, and the CRP level is 200 mg/L. The patient reports experiencing numbness and tingling of the distal right hand and wrist area. Urinalysis yields normal results, but the creatinine concentration is 2.2 mg/dL. Tests for ANA, ANCA, and rheumatoid factor (RF) yield negative results.
Which of the following is the most likely diagnosis?
Image courtesy of Medscape.
Answer: D. PAN.
Tender, hyperpigmented, firm subcutaneous nodules with a background of livedo reticularis, common in cutaneous PAN, are shown. Common features of PAN include fever, arthralgia, abdominal pain, nausea and vomiting, diarrhea, asymmetric peripheral neuropathy, arthralgia, nodular skin lesions, and a lacy erythematous rash called livedo reticularis.[11] Skin manifestations are common in the vasculitic disorders. In addition to the tender erythematous skin nodules seen in PAN, dermatologic findings include purpura, petechiae, papulopustular lesions, erythema nodosum, Raynaud phenomenon, lower-extremity swelling, and recurrent oral and genital ulcerations that heal with scarring.
What is the most common form of vascular anomaly noted on angiography in PAN?
Image courtesy of Medscape.
Answer: A. Microaneurysm.
Microaneurysms may appear in many vascular territories, classically the renal arteries. A renal angiogram demonstrates multiple microaneurysms (arrows), characteristic of PAN, in the upper pole of the right kidney. Glomerulonephritis is not associated with PAN, but renal infarction or compromise may result from major renal artery vasculitis.[12] ANCA is usually negative.
Image courtesy of Medscape.
A 62-year-old white man presents with a diffuse skin rash with palpable purpura. He has noted low-grade fever, myalgia, arthralgia, and an 8-lb weight loss. Apart from a productive, blood-tinged cough, he has no other symptoms. Physical examination is notable for a blood pressure (BP) of 170/95 mm Hg and the rash shown in the slide. Urinalysis shows proteinuria and granular, RBC, and white blood cell (WBC) casts. Creatinine is 3.2 mg/dL. P-ANCA is positive.
Skin lesions are most likely to be found in vasculitis involving vessels of what size?
Image courtesy of Medscape.
Answer: E. MPA.
MPA is a small- to medium-sized-vessel vasculitis consistent with hypersensitivity vasculitis (shown).[13] Focal or diffuse glomerulonephritis is a frequent finding, as are pulmonary infiltrates. Ear, nose, and throat manifestations are not considered to be clinical symptoms of MPA but are very frequently found. ANCA is usually positive and can be either perinuclear (P-ANCA) or cytoplasmic (C-ANCA). Aggressive immunosuppressive treatment typically results in remission.
Image courtesy of Medscape.
A 42-year-old white man presents to the ED because of a purpuric skin rash (shown) and an acute foot drop. He has a 2-year history of asthma and sinusitis treated with inhaled and oral steroids but no history of rash or neurologic disease. He reports fatigue, a 10-lb weight loss, a cough occasionally productive of bloody sputum, and arthralgia without objective arthritis. His past medical history is otherwise negative. Initial laboratory testing includes a CBC with a WBC count of 10,500/µL (72% neutrophils, 16% lymphocytes, 12% eosinophils). Urinalysis is normal. BUN, creatinine, and glucose are within normal limits.
Image courtesy of Medscape.
A chest radiograph was obtained on an outpatient basis 1 week before presentation (shown). A subsequent radiograph demonstrates no abnormalities. A tissue biopsy is scheduled, and serologic tests are ordered.
In view of the clinical presentation and the study results to this point, which of the following is the suspected diagnosis?
Image courtesy of Medscape.
Answer: A. Eosinophilic granulomatosis with polyangiitis (EGPA; also referred to as Churg-Strauss syndrome).
EGPA is characterized on biopsy by small-vessel granulomatous vasculitis with a pronounced eosinophilic infiltrate.[14,15] Note the eosinophilic infiltrate surrounding small vessels in this lung biopsy. This form of vasculitis is generally preceded by asthma and often by sinusitis. In most cases, other features (eg, purpuric skin rash, eosinophilia, and transient pulmonary infiltrates) develop within 3 years of the onset of asthma. ANCA testing is positive in 40% of patients. ANCA in EGPA reacts to myeloperoxidase, which is concentrated in the perinuclear area (ie, P-ANCA).
Image courtesy of Wikimedia Commons.
Angiography is used to assess for vasculitis of large or medium-sized vessels. Magnetic resonance angiography (MRA) is diagnostic in large- or medium-sized-vessel primary angiitis of the central nervous system (PACNS); patients with suspected small-vessel angiography-negative PACNS may require tissue biopsy. Findings in PAN include "beading" of vessels caused by alternating areas of vascular narrowing and dilatation. Histopathologic patterns of vessel inflammation include lymphocytic/giant cell–mediated large-vessel vasculitis (eg, giant cell arteritis); necrotizing vasculitis (eg, PAN or Kawasaki disease); antibody-mediated small-vessel vasculitis (ie, ANCA-positive vasculitides); and antibody (IgA) deposition vasculitis (Henoch-Schönlein purpura).
Chest radiography is indicated to screen for pulmonary involvement. CT of the sinuses may show turbinate mucosal thickening and erosive changes associated with sinusitis (GPA). Sinus CT is also useful for evaluating orbital pseudotumor (GPA or MPA).
Chest CT can detect the following[16]:
When performed by skilled imagers, two-dimensional echocardiography has 100% sensitivity and approximately 90% specificity for detecting aneurysms of the proximal coronary arteries in patients with Kawasaki disease. CT or MRI of the head may show acute ischemia or hemorrhage in patients with central nervous system symptoms.
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