Large-sized vessel vasculitis with arthralgia

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Eiman Ghaffarpasand, M.D. [2]

Abbreviations: ABG= Arterial blood gas, ANA= Antinuclear antibody, ANP= Atrial natriuretic peptide, ASO= Antistreptolysin O antibody, BNP= Brain natriuretic peptide, CBC= Complete blood count, COPD= Chronic obstructive pulmonary disease, CRP= C-reactive protein, CT= Computed tomography, CXR= Chest X-ray, DVT= Deep vein thrombosis, ESR= Erythrocyte sedimentation rate, HRCT= High Resolution CT, IgE= Immunoglobulin E, LDH= Lactate dehydrogenase, PCWP= Pulmonary capillary wedge pressure, PCR= Polymerase chain reaction, PFT= Pulmonary function test.

Diseases Clinical manifestations Para-clinical findings Gold standard Additional findings
Symptoms Physical examination
Lab Findings Imaging Histopathology
Headache Fever Weight loss Arthralgia Claudication Bruit HTN Focal neurological disorder Biomarker CBC ESR Other CT scan Angiography Ultrasound/ Echocardiography Other
Large-Vessel Vasculitis Giant cell arteritis[1] + - + +/- - - - +/- Pentraxin 3 (PTX3) Normal ↑ ↑CRP Stenosis, Occlusion, Dilatation Aneurysm - Mural inflammation in MRA Granulomatous inflammation of arteries Biopsy  Jaw pain and claudication
Systemic disease Fibromuscular dysplasia[2] + - +/- + + + + +/- Transforming growth factor β (TGF-β) Normal ↑ ↑ Cr or BUN Alternating stenosis and dilatations in CT angiography Stenosis in the renal arteries Luminal narrowing alternating with dilatation (Beads sign) Focal concentric, long-segment tubular stenosis or outpouching in MRA Fibrodysplastic changes, Collagen deposition Digital subtraction angiography (DSA)  Spontaneous coronary artery dissection (SCAD)
Ehlers-Danlos syndrome[3] - - - + +/- +/- - +/- TGF-β Normal Normal Cultured skin fibroblasts Multiple vascular segments with aneurysms and dissections Dissection of the posterolateral branch of the left circumflex coronary artery (LCx) Visceral arteries abnormality Vascular abnormalities in MRA Thin and rare collagen bundles in the dermis History and physical examination Bleeding, Bruisability, Heart murmur
Polymyalgia rheumatica (PMR)[4] + - +/- + - - - - Plasma fibrinogen Normocytic, normochromic anemia ↑ CRP Periodontoid localization of calcification Vessel wall thickening, Increased mural contrast enhancement Subacromial or subdeltoid bursitis High F-FDG accumulation around the joints in FDG PET-CT Small angular fibers, Pyknotic nuclear clumps, or target-targetoid fibers - Joint stiffness, Fatigue
Amyloidosis[5] +/- - - +/- +/- - + + Immunoglobulin light chain(Amyloid) Anemia Normal ↑ Cr or BUN,

↑ ALT or AST

Diffusely hypoattenuating and enlarged liver Amyloid deposition in the media and adventitia of small arteries  Solid organs increased echogenicity Tc-DPD for cardiac amyloid deposits Extracellular deposition of fibrillar proteins Biopsy Cardiomegaly, Dyspnea

References

  1. ↑ Calvo-Romero JM (2003). "Giant cell arteritis". Postgrad Med J. 79 (935): 511–5. PMC 1742823. PMID 13679546.
  2. ↑ Plouin PF, Perdu J, La Batide-Alanore A, Boutouyrie P, Gimenez-Roqueplo AP, Jeunemaitre X (2007). "Fibromuscular dysplasia". Orphanet J Rare Dis. 2: 28. doi:10.1186/1750-1172-2-28. PMC 1899482. PMID 17555581.
  3. ↑ Gazit Y, Jacob G, Grahame R (2016). "Ehlers-Danlos Syndrome-Hypermobility Type: A Much Neglected Multisystemic Disorder". Rambam Maimonides Med J. 7 (4). doi:10.5041/RMMJ.10261. PMC 5101008. PMID 27824552.
  4. ↑ Michet CJ, Matteson EL (2008). "Polymyalgia rheumatica". BMJ. 336 (7647): 765–9. doi:10.1136/bmj.39514.653588.80. PMC 2287267. PMID 18390527.
  5. ↑ Baker KR, Rice L (2012). "The amyloidoses: clinical features, diagnosis and treatment". Methodist Debakey Cardiovasc J. 8 (3): 3–7. PMC 3487569. PMID 23227278.

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