General Presentation of the Vasculitides

, David G. I. Scott² and Chetan Mukhtyar²

(1)

Department of Rheumatology, Ipswich Hospital NHS Trust, Ipswich, UK

(2)

Department of Rheumatology, Norfolk and Norwich University Hospital, Norwich, UK

3.1 Introduction

Primary systemic vasculitis can involve the vasculature of any organ-system. Depending on the calibre of the vessels involved and the organs affected, vasculitis can present in a myriad of ways to a number of specialties. There are no diagnostic criteria or pathognomonic laboratory investigations for systemic vasculitis. The application of classification criteria in clinical practice have been disappointing and should be avoided. The classification criteria of the American College of Rheumatology had a positive predictive value of 17–29 % in a cohort of patients with a differential diagnosis of systemic vasculitis [1]. The Chapel Hill nomenclature is similarly unsatisfactory [2]. Histopathology can be diagnostic, but has variable diagnostic yield depending on the skill of the operator, method of sampling, and the organ biopsied [3, 4].

3.2 Pattern Recognition

In the absence of assistance from laboratory tests or diagnostic criteria, recognition of clinical patterns supported by laboratory tests is of value in the diagnosis of systemic vasculitis. Table 3.1 provides examples of presentation patterns of specific vasculitic syndromes [5]. The table illustrates the value of a structured clinical interview and examination at presentation and follow-up. The rarity of systemic vasculitis and the multisystem nature of the pathology often cause delay in diagnosis. The Birmingham Vasculitis Activity Score is a validated clinical tool to quantify disease activity in patients with systemic vasculitis [6]. It is a single A4 sheet listing common or important manifestations of systemic vasculitis. The author uses it to quantify disease activity in clinical practice, but it also serves as an aide memoire of clinical manifestations to check for patients with a suspicion of systemic vasculitis (Table 3.2).

Table 3.1

Common clinical patterns of vasculitis

Examples of presentation	Possible diagnosis
Recurrent sinonasal disease with or without septal perforation, orbital involvement (radiological or clinical)	Granulomatosis with polyangiitis
A pulmonary syndrome on the background of chronic poorly controlled asthma with peripheral eosinophilia with or without a peripheral neuropathy	Eosinophilic granulomatosis with polyangiitis
Pulmonary-renal syndrome: microscopic haematuria, hypertension, renal impairment, dyspnoea, haemoptysis	Granulomatosis with polyangiitis or microscopic polyangiitis
Vasculitic rash, ulcer, neuropathy on the background of hepatitis or the presence of liver abnormalities at presentation	Cryoglobulinaemic vasculitis
Purpuric lesions mainly on the extensor surface of the lower limbs including gluteal involvement with or without microscopic hematuria, arthritis and abdominal pain	IgA vasculitis
Multiple constitutional symptoms, leg ulcers, orchitis, abdominal pain, hypertension without evidence of glomerular or alveolar involvement	Polyarteritis nodosa
A new onset headache in individuals above the age of 50 with temporal artery or scalp tenderness and raised ESR	Giant cell arteritis
New-onset vascular claudication in any limb with peripheral pulse abnormalities in an individual less than 40 years of age	Takayasu’s arteritis
Acute onset of fever, desquamating rash and mucocutaneous lymphadenopathy in a toddler	Kawasaki’s disease
Orogenital ulceration, hypopyon, uveitis, arthritis in a young male of Mediterranean descent	Behçet’s disease
Constitutional symptoms, raised inflammatory markers, patchy aortic and/or periaortic inflammation with hepato-biliary or pancreatic involvement	IgG4 related disease