Approach to vasculitis: Clinical sciences

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Introduction 0:00–0:38

Vasculitis refers to inflammation of blood vessels, which can result in vessel wall damage, bleeding, and ischemia of affected organs.
Based on the size of the affected blood vessels, large-vessel vasculitis affects vessels like the aorta and its branches; medium-vessel vasculitis primarily affects major visceral arteries like renal and mesenteric arteries; small-vessel vasculitis mostly affects arterioles and capillaries; and variable-vessel vasculitis can affect vessels of any size.
Now, if your patient presents with a chief concern suggesting vasculitis, first perform an ABCDE assessment to determine if they are unstable or stable.

Unstable 0:38–1:34

If unstable, stabilize their airway, breathing, and circulation. Obtain IV access and put your patient on continuous vital sign monitoring including heart rate, blood pressure, and pulse oximetry; as well as cardiac telemetry.
If needed, provide supplemental oxygen. Now here’s a clinical pearl to keep in mind!
Vasculitides that commonly present as unstable include ANCA-associated small-vessel vasculitis, which can cause diffuse alveolar hemorrhage; and anti-glomerular basement membrane or anti-GBM disease, which can cause damage to the glomerular basement membrane.
Together, these conditions can result in pulmonary-renal syndrome, which can lead to respiratory and renal failure! Now, let’s go back to the ABCDE assessment and discuss stable patients.

Stable1:34–3:06

In this case, obtain a focused history and physical examination; and order labs, including CBC, CMP, ESR, CRP, and urinalysis.
Your patient will typically report constitutional symptoms, including fever, fatigue, anorexia, and weight loss; as well as epistaxis, limb claudication, and Raynaud phenomenon.
Ask about symptoms that could indicate involvement of specific organ systems. These patients may experience wheezing or hemoptysis; rash or skin discoloration; sensory disturbances like vision loss or limb weakness; or discolored urine or anuria.
The physical exam may reveal an elevated temperature, as well as vascular findings like decreased, asymmetric pulses with vessel tenderness to palpation.
You may notice organ-specific findings like nasal crusting or abnormal lung sounds; palpable purpura or skin ulceration; sensory deficits, such as numbness; or motor deficits like foot drop.
As far as labs go, the CBC usually reveals leukocytosis with neutrophilic predominance, thrombocytosis, and low hemoglobin and hematocrit.
The CMP might show elevated BUN and creatinine; the ESR and CRP are generally elevated, and the urinalysis may reveal hematuria or proteinuria.
With these findings, consider vasculitis, and order labs to confirm the diagnosis and determine the underlying cause. Start by ordering the antineutrophil cytoplasmic antibodies, or ANCA, subtypes MPO-ANCA and PR3-ANCA.

Additional Labs 3:06–3:47

Next, order antinuclear antibody or ANA and anti-glomerular basement membrane, or anti-GBM antibody, as well as serum cryoglobulins and rheumatoid factor, or RF.
Also check the complement levels, serum immunoglobulins, and serologies for hepatitis B, hepatitis C, and HIV. Now, let’s start with large-vessel vasculitis, which includes Takayasu arteritis and giant cell arteritis.

Large-Vessel Vasculitis Takayasu Arteritis 3:47–4:35

Takayasu arteritis is more common in biological females under the age of 50. Patients usually report claudication, or pain in the limbs during movement.
The physical exam will reveal unequal blood pressure between arms and diminished pulses in the extremities. Arterial bruits may also be present.
With these findings, consider Takayasu arteritis, and order MR angiography of the arterial tree. If CT or MR angiography reveals luminal narrowing or vascular wall thickening of large vessels such as the aorta or its primary branches, this confirms a diagnosis of Takayasu arteritis.
On the flip side, giant cell arteritis is more common in patients over 50 years of age, and is often associated with polymyalgia rheumatica.

Giant Cell Arteritis 4:35–5:25

These individuals typically report unilateral headaches and jaw claudication while chewing. Some patients may also report vision loss.
The physical exam often reveals an enlarged, tender, and nodular temporal artery. At this point, consider giant cell arteritis.
To confirm, obtain either a temporal artery biopsy or an ultrasound of the temporal artery with Doppler. If the biopsy reveals transmural inflammatory infiltration with giant cells, or the ultrasound shows a circumferential thickening of the vascular wall around the lumen, or the classic halo sign, diagnose giant cell arteritis.
Alright, switching gears to medium-vessel vasculitis, which includes polyarteritis nodosa and thromboangiitis obliterans.

Polyarteritis Nodosa 5:25–6:26

Individuals with polyarteritis nodosa typically report abdominal and extremity pain, and weakness. The physical exam reveals livedo reticularis, characterized by lacy, mottled skin discoloration often in combination with skin ulcers and nodules.
Finally, you might notice concurrent sensorimotor deficits in various locations, such as sciatic and radial neuropathy, which is often referred to as mononeuritis multiplex.
Labs may reveal hematuria with no red blood cell casts, and in some cases, positive hepatitis B serology. With these findings, consider polyarteritis nodosa, and order a renal or mesenteric angiography.
If it shows microaneurysms and focal vessel narrowing and occlusion, diagnose polyarteritis nodosa. On the other hand, patients with thromboangiitis obliterans are almost exclusively younger than 50 years old, with heavy tobacco use.

Thromboangiitis Obliterans 6:26–7:09

The physical exam typically reveals digital ulcers, and sometimes gangrene. With these findings, consider thromboangiitis obliterans and obtain an ankle-brachial index or wrist-brachial index of the involved extremities.
You may also need an angiogram of the extremities. If the ankle-brachial or wrist-brachial index is abnormal, or the angiography shows segmental arterial occlusions with collateral vessel formation which gives a classic ‘corkscrew’ appearance, diagnose thromboangiitis obliterans!
Alright, moving on to small-vessel vasculitis, start by assessing the presence of ANCA. If ANCA is positive, consider an ANCA-associated vasculitis, like granulomatosis with polyangiitis, or GPA; microscopic polyangiitis, or MPA; and eosinophilic granulomatosis with polyangiitis, or EGPA.

Small-Vessel Vasculitis 7:09–7:15

ANCA-Associated Vasculitis 7:15–7:32

Alright, let’s start with GPA and MPA. These patients generally have a history of shortness of breath and hemoptysis, while their physical exam usually reveals abnormal lung sounds.

GPA/MPA 7:32–8:28

Labs will show elevated BUN and creatinine, proteinuria, and hematuria with red blood cell casts. With these findings, consider either GPA or MPA, and obtain a biopsy of the affected tissue.
Now, let’s say your patient has an additional history of sinusitis and epistaxis, the physical exam reveals a saddle-nose deformity, and labs show the presence of PR3-ANCA.
If the biopsy reveals necrotizing granulomatous inflammation, diagnose GPA. On the other hand, if your patient has no upper respiratory involvement, labs show MPO-ANCA, and biopsy reveals necrotizing non-granulomatous inflammation, diagnose MPA.
Next up is EGPA, which is usually associated with new-onset asthma or rhinosinusitis. The physical exam will reveal mononeuritis multiplex and skin nodules, with labs showing eosinophilia, possibly in combination with positive MPO-ANCA.

EGPA 8:28–9:00

With these findings, consider EGPA, and obtain a biopsy, usually from the skin or kidneys. If the biopsy sample shows microgranulomas with eosinophilic infiltrates, diagnose EGPA.
Okay, let’s discuss situations when ANCA is negative. In this case, you should consider immune-complex vasculitis, which is caused by the deposition of immunoglobulins and complements into the vessel walls.

Immune Complex Vasculitis 9:00–9:14

Let’s start with anti-GBM disease! These patients primarily report hemoptysis, while the physical exam typically reveals elevated blood pressure and peripheral edema.

Anti-GBM Disease 9:14–9:51

Labs will reveal elevated BUN and creatinine; the presence of anti-GBM antibodies; proteinuria, and hematuria with red blood cell casts.
With these findings, consider anti-GBM disease, and obtain a renal biopsy. If the results reveal linear IgG deposits along the glomerular basement membrane, diagnose anti-GBM disease.
Next up is IgA vasculitis! Your patient typically reports abdominal and joint pain, and the physical exam reveals palpable purpura.

IgA Vasculitis 9:51–10:25

Labs often reveal elevated BUN and creatinine, elevated IgA levels, and hematuria with or without red blood cell casts. In this case, consider IgA vasculitis, and obtain a skin or renal biopsy.
If the biopsy shows leukocytoclastic vasculitis with IgA and C3 deposition, diagnose IgA vasculitis! Next, let’s discuss cryoglobulinemic vasculitis!

Cryoglobulinemic Vasculitis 10:25–10:59

These patients typically report fatigue and joint pain, and their physical exam will likely show palpable purpura and peripheral neuropathy.
Labs typically reveal positive cryoglobulins, rheumatoid factor, decreased C4, and possibly positive serologies for hepatitis C, hepatitis B, or HIV.
In some cases, you might notice hematuria and proteinuria. With these findings, diagnose cryoglobulinemic vasculitis.
Lastly, let’s have a look at variable-vessel vasculitis, starting with Behçet disease! If your patient reports recurrent oral and genital ulcers, with the physical exam showing uveitis, skin lesions, and a positive pathergy test, diagnose Behçet disease.

Variable-Vessel Vasculitis Behçet Disease 10:59–11:33

Now here’s a clinical pearl! The pathergy test is performed by inserting a sterile needle into the skin and examining the area after one to two days.
The test is positive when a small red bump forms at the site of the needle insertion. On the other hand, patients with Cogan syndrome typically present with eye redness and pain, or photophobia, and a history of sensorineural hearing loss in combination with vertigo.

Cogan Syndrome 11:33–11:50

If the physical exam shows corneal inflammation and ataxia, diagnose Cogan syndrome. All right, as a quick recap… Vasculitis refers to inflammation of blood vessels, which can result in vessel wall damage, bleeding, and ischemia of affected organs.

Review 11:50–12:22

Based on the size of the affected blood vessels, large-vessel vasculitis affects vessels like the aorta and its branches; medium-vessel vasculitis primarily affects major visceral arteries like renal and mesenteric arteries; small-vessel vasculitis mostly affects arterioles and capillaries; and variable-vessel vasculitis can affect vessels of any size.
Approach to vasculitis: Video, Steps, and Uses | Osmosis