|Year : 2014 | Volume
| Issue : 2 | Page : 201-204
A case of small vessel vasculitis
Madhulika Mahashabde, Harshad Patil
Department of Internal Medicine, Padmashree Dr. D. Y. Patil Medical College, Hospital and Research Centre, Dr. D. Y. Patil Vidyapeeth, Pimpri, Pune, Maharashtra, India
|Date of Web Publication||4-Feb-2014|
Department of Internal Medicine, Padmashree Dr D. Y. Patil Medical College Pimpri, Pune
Source of Support: None, Conflict of Interest: None
Anti-neutrophil cytoplasmic antibodies (ANCA) associated vasculitis is the most common primary systemic small-vessel vasculitis occurring in adults. Although the etiology is not always known, the incidence of vasculitis is increasing, with a variability of clinical expression.
We are reporting a case of un-specified small vessel vasculitis, which was diagnosed on the basis of positive perinuclear anti neutrophil cytoplasmic antibodies (ANCA) P MPO done by Enzyme Linked Immunosorbent Assay (ELISA).
Keywords: Acute vasculitis, anti neutrophil cytoplasmic antibodies P (MPO), Enzyme Linked Immunosorbent Assay
|How to cite this article:|
Mahashabde M, Patil H. A case of small vessel vasculitis. Med J DY Patil Univ 2014;7:201-4
| Introduction|| |
Vasculitis may affect the large, medium, or small blood vessels. Small-vessel vasculitis may be further classified as ANCA-associated or non-ANCA associated vasculitis. ANCA associated small-vessel vasculitis includes microscopic polyangiitis, Wegener's granulomatosis, Churg-Strauss syndrome, and drug-induced vasculitis. Vasculitis may be confined to single organ such as the skin or may simultaneously involve several organs.
Vasculitis syndrome as a group has heterogenecity as well as overlap of clinical features among them.
| Case Report|| |
A 53 year old menopausal, obese (weight 90 kg) female presented with complaint of polyarthalgia involving lower limb since three to four months, difficulty in walking, climbing stairs, getting up from squatting position associated with bilateral lower limb pain and paresthesia since 3 to 4 weeks. In due course, she developed rash involving bilateral feet since five days. No history of fever, drugs, hemoptysis, GI bleed, hematuria, oliguria, breathlessness was noted.
Patient was diagnosed with type II diabetes mellitus and hypothyroidism since seven years and was well controlled with the oral drugs.
On examination, her pulse rate was 80 beats / min, blood pressure 130/90 mm Hg; pallor was present; there was no lymphadenopathy and no peripheral edema. Rashes were noted over dorsum of both feet. These rashes were erythematous, palpable, and tender [Figure 1], [Figure 2], [Figure 3].
|Figure 1: Purplish, erythematous, palpable petechial to confluent rash involving Bilateral lower legs, feet, soles, which was painful.|
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|Figure 2: Purplish, erythematous, palpable petechial to confluent rash involving Bilateral lower legs, feet, soles, which was painful.|
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|Figure 3: Purplish, erythematous, palpable petechial to confluent rash involving Bilateral lower legs, feet, soles.|
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Systemic examination of nervous system revealed tingling sensation over both lower limbs; sensory system examination revealed reduced sensation of touch (crude touch and fine touch), pain and temperature sensation in both lower limb, reduced ankle jerk, and proximal muscle weakness in lower limbs. Other examination and other systems were normal.
Laboratory and special investigations
Hemoglobin was 9.1 gm%,
WBC - 14900/cmm and platelet count of 5.2 lakhs/cmm,
ESR was 132 mm at the end of 1 st hour.
Reduced iron levels of 46 mcg/dl and transferrin saturation of 11.53%. Liver function test were normal and renal function test revealed serum urea 30 mg/dl and serum creatinine 1.3 mg/dl.
TSH was 2.24 uIU/ml; microsomal TPO antibody was negative; thyroglobulin antibody was positive (11.92).
RA factor was 142.6 IU/ml, but anti CCP antibodies were negative. Parathyroid hormone levels were normal. Chest X-ray showed bilateral lower zone haziness.
DXA bone scan was suggestive of osteopenia.
Blood glucose levels were normal, no evidence of diabetic retinopathy on fundus examination.
Patient underwent nerve conduction study with suggested generalized motor mixed (demyleinating and axonal neuropathy in both sural nerve and right median nerve).
ANA and anti Ds DNA was negative; Coombs test was negative; LE cells / Lupus anticoagulant was negative.
D dimer was 12120 ng/ml; so pulmonary angiography was advised which was normal, following which work up for deep vein thrombosis was done which was normal. Complement C3 and C4 were normal.
ANCA antibodies were sent; ANCA C(PR3) was negative (3.24 u/ml); ANCA P(MPO) was positive (62.3 u/ml) by EIA method.
2D echocardiography was suggestive of tricuspid regurgitation with moderate pulmonary hypertension.
X-ray chest was normal.
Following which a biopsy of the rash was examined, which showed skin without subcutaneous fat, dermis containing heavy perivascular infiltrates of neutrophil, few lymphocytes and eosionphils, all suggestive of acute vasculitis [Figure 4].
|Figure 4: The dermis contains heavy perivascular infiltrates of neutrophils, few lymphocytes and eosinophils. No granuloma.|
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Based on the diagnosis of acute vasculitis, patient was started on treatment with Corticosteroids 1 mg/kg body weight given for 3 months and tapered off over 3 months, Azathioprine 150 mg /day for 1 year and tapered over next 6 months. Plasma glucose levels were controlled by insulin.
Follow up of case
On follow up after 10 days, the skin rashes were resolved, with continued treatment, presently patient has improved hematologically, and neuropathy and proximal myopathy has improved.
ANCA P (MPO), RA factor, CRP repeated after 6 months following treatment were negative with reduced ESR, which was indicative of remission of disease following treatment.
Patient is now under regular follow up.
| Discussion|| |
Vasculitis is a clinicopathologic process characterized by inflammation of and damage to blood vessels. The vessel lumen is usually compromised leading to ischemia of tissue supplied by involved vessel.
According to the size of the vessel affected, vasculitis can be classified into 
- Large vessel: Polymyalgia rheumatica, Takayasu's arteritis, temporal arteritis
- Medium vessel: Buerger's disease, cutaneous vasculitis, Kawasaki disease, polyarteritis nodosa
- Small vessel: Behçet's syndrome, Churg Strauss syndrome, cutaneous vasculitis, Henoch Schönlein purpura, microscopic polyangiitis, Wegener's granulomatosis.
ANCA are antibodies directed against certain proteins in the cytoplasmic granules of neutrophil and monocytes. These autoantibodies are present in high percentage of patients with Wegener's granulomatosis, microscopic polyangiitis, and Churg-Strauss syndrome. They share the presence of ANCA and small vessel vasculitis, so collectively known as ANCA associated vasculitis. 
In 1982, antibodies directed against neutrophil cytoplasmic antigens were first described in patients with pauci immune glomerulonephritis. ANCA are antibodies directed against certain proteins in cytoplasmic granules of neutrophil and monocytes. These autoantibodies are present in high percentage of patients with Wegener's granulomatosis, microscopic polyangiitis, Churg Strauss syndrome.
The diagnosis of ANCA associated vasculitis is made on the basis of clinical findings, biopsy of involved organs, and presence of ANCA. Testing for ANCA includes indirect immunofluorescence and antigen specific enzyme linked immunosorbent assay, which provides 99% sensitivity and 70% specificity. 
Two major patterns of staining include cytoplasmic ANCA (C-ANCA), perinuclear ANCA (P-ANCA). C-ANCA is antibody to proteinase 3; P ANCA is antibody to myeloperoxidase. So, a more specific term proteinase 3 ANCA and myeloperoxidase ANCA are now in use. 
Vasculitis presents with clinical features of ,
- General symptoms: Fever, weight loss
- Skin: Palpable purpura, livedo reticularis
- Muscles and joints: Myalgia or myositis, arthralgia or arthritis
- Nervous system: Mononeuritis multiplex, headache, stroke, tinnitus, reduced visual acuity, acute visual loss
- Heart and arteries: Myocardial infarction, hypertension, gangrene
- Respiratory tract: Nose bleeds, bloody cough, lung infiltrates
- GI tract: Abdominal pain, bloody stool, perforations
- Kidneys: Glomerulonephritis.
Case reported above had clinical spectrum with skin involvement as palpable purpura, nervous system involvement as peripheral neuropathy and proximal myopathy and hematological involvement, gastrointestinal involvement in the form of vague abdominal pain. Although our case had no renal and pulmonary involvement, skin lesions and skin biopsy with positive ANCA P(MPO) were diagnostic for small vessel vasculitis.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]