|Year : 2019 | Volume
| Issue : 2 | Page : 101-103
A unique case of systemic lupus erythematosus with medium- and large-vessel vasculitis
Subbalaxmi V S. Malladi1, Sumanth Vittamraj1, Anu Kapoor2, Naval Chandra1, Liza Rajasekhar3
1 Department of General Medicine, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India
2 Department of Radiology and Imageology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India
3 Department of Rheumatology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India
|Date of Web Publication||11-Nov-2019|
Subbalaxmi V S. Malladi
Professor, Department of General Medicine, Nizam's Institute of Medical Sciences, Hyderabad, Telangana
Source of Support: None, Conflict of Interest: None
Medium and large-vessel vasculitis in association with SLE is distinctly uncommon and is limited to occasional case reports only. We present a case of 40 year old female who presented with complaints of left sided pleuritic chest pain of 10 days duration. She was diagnosed to have hypertension 1 year ago which was treatment resistant. In present admission she had accelerated hypertension for which she was evaluated systematically. She was diagnosed to have SLE with Bilateral Renal artery stenosis and stenosis of other visceral vessels. Literature search revealed very few cases of SLE presenting with features of medium and large vessel vasculitis. Large vessel involvement in SLE is associated with high disease activity and therefore early recognition of this rare entity is essential.
Keywords: Large-vessel vasculitis, systemic lupus erythematosus, vasculitis
|How to cite this article:|
S. Malladi SV, Vittamraj S, Kapoor A, Chandra N, Rajasekhar L. A unique case of systemic lupus erythematosus with medium- and large-vessel vasculitis. J Clin Sci Res 2019;8:101-3
|How to cite this URL:|
S. Malladi SV, Vittamraj S, Kapoor A, Chandra N, Rajasekhar L. A unique case of systemic lupus erythematosus with medium- and large-vessel vasculitis. J Clin Sci Res [serial online] 2019 [cited 2019 Dec 9];8:101-3. Available from: http://www.jcsr.co.in/text.asp?2019/8/2/101/270747
| Introduction|| |
Systemic lupus erythematosus (SLE) is a multisystem disease of autoimmune origin. Vasculitis is often seen with SLE but is usually limited to small vessels alone. The primary pathology in SLE-related vasculitis is leukocytoclastic vasculitis. Medium- and large-vessel vasculitis in association with SLE is distinctly uncommon and is limited to occasional case reports only. In addition, reports of medium-vessel vasculitis with SLE generally describe the involvement of a single-vessel bed. We report a rare case of extensive vasculitis involving medium-sized vessels and large vessels in a patient with SLE. This case also illustrates the need for proper evaluation of young patients with hypertension before labelling them as resistant hypertension, thus avoiding unnecessary medications, and also averts target organ damage.
| Case Report|| |
A 40-year-old female homemaker, resident of Kakinada, Andhra Pradesh, has presented with complaints of pain localised to the left anterior chest for 10 days. Pain was aggravated on deep inspiration. She also complained of pain and stiffness of all small joints of hands and feet, bilateral wrist joints and elbow joints. History of early morning stiffness of more than 1 hr was present. She was diagnosed to have hypertension 1 year ago with no response to 3 antihypertensive agents. There is no history of rash, oral ulcers, alopecia and photosensitivity. On examination, the patient was moderately built and nourished, pallor was present and there was no icterus or lymphadenopathy or pedal oedema. Temperature was 37°C, pulse rate was 88/min regular rhythm, unequal in volume (left < right) and blood pressure was 180/100 mmHg in both the upper limbs and 190/100 mmHg in the lower limbs. Rest of physical examination was unremarkable. Investigations showed a normocytic, normochromic anaemia (haemoglobin – 8.9 mg/dL), leucopenia (total leucocyte count – 3500 cells/mm3) and normal platelet count. Renal and liver function tests, serum electrolytes and urine examination were normal. Radiograph of both the hands with wrists showed diffuse osteopenia without any bone erosion. Direct Coomb's test was positive. C3 was low normal at 69 mg/dL (65–120 mg/dL), whereas C4 was normal at 15 mg/dL (10–40 mg/dL). Anti-nuclear antibody was 4+ positive and showed a speckled immunofluorescence pattern. Anti-dsDNA was positive: 2.30 (normal <1.1). Anti-smith antibody was also positive: 1.8 (normal <1.1). IgM rheumatoid factor, anti-cyclic citrullinated peptide Ab and antiphospholipid antibodies were negative. Hepatitis B surface antigen, HIV, anti-hepatitis C virus and antineutrophil cytoplasmic antibody were negative. Ultrasonography of chest showed bilateral minimal pleural effusion. Doppler study of neck vessels showed arterial wall thickening, irregularity and luminal narrowing in the left subclavian artery. Renal vessel doppler study mild arterial wall thickening with accelerated time in bilateral renal arteries at the origin and infrarenal aorta. Transthoracic echocardiography revealed no abnormality. Computed tomography aortogram showed narrowing at the origin of the left subclavian artery, superior mesenteric artery, celiac artery, bilateral renal arteries at the origin and inferior mesenteric artery as shown in [Figure 1]. Minimal circumferential wall thickening in the infrarenal segment of the aorta was also noted. The patient was considered to have SLE with medium-sized vessel vasculitis of visceral, renal, limb vessels and large-vessel vasculitis of the infrarenal aorta. In view of the generalised vasculitis, she was started on intravenous (IV) methylprednisolone 1 g for 3 days, followed by oral prednisolone 1 mg/kg slowly tapered to 10 mg/day dose. Pulse IV cyclophosphamide 1 g/m2 once a month was given for the initial 6 months, and thereafter, it was reduced to 3 monthly pulses. The patient had to be put on three classes of antihypertensives initially; however, after 2 months, she required only one class of antihypertensive.
|Figure 1: (1) Left subclavian artery thickening, (2) infrarenal aorta wall thickening, (3) right renal artery stenosis, (4) left renal artery stenosis|
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| Discussion|| |
Vasculitis in SLE affects 10% of patients and is usually small-vessel vasculitis. The primary pathology is leukocytoclastic vasculitis and major organs affected are skin, nervous system, gastrointestinal tract and eyes. Medium-sized vessel vasculitis is not well known in SLE and is limited to case reports only. The largest case series in this respect was published in 1991 by Weiner and Allen. They reported a case of central nervous systems vasculitis involving both anterior and posterior circulations in a case of SLE. They also compiled a total of 11 case reports from 1954 to 1989 and published a review of this literature. Vasculitis was described based on either angiography or histopathologic examination. The prognosis was ominous despite aggressive treatment, although a role of oral and IV cyclophosphamide was proposed. Suzuki et al., in 1990, reported a case of cerebral and systemic necrotising vasculitis developing during pregnancy in a case of SLE. The diagnosis of necrotising vasculitis was confirmed on autopsy. Another similar case was reported by Mitsias and Levine in 1994. Irlapati et al., in 2016, reported four cases of SLE with large-vessel vasculitis which predominantly involved aorta and subclavian vessels. They had reported that disease activity was significantly higher in these cases.
Our patient was diagnosed with SLE as she satisfied 7 out of 17 Systemic Lupus International Collaborating Clinics criteria which included three clinical criteria and four immunological criteria. She was evaluated as a case of stroke in young and hypertension in young. Clinically, unequal pulse volume in both the upper limbs provided a clue for a possibility of systemic vasculitis as the patient did not have telltale features of SLE such as arthritis, cutaneous lesions, oral ulcers, alopecia and photosensitivity. Rheumatoid arthritis (RA) was a possible differential initially in this patient because she had presented with symmetrical small-joint arthralgias of the hands, lack of constitutional symptoms and vasculitis. Vasculitis of small and medium vessels is sometimes seen, especially in seropositive RA, and can involve any organ system and generally has a poor prognosis. It is usually seen in patients with long-standing RA.
There are very few cases of SLE presenting with features of large-vessel vasculitis in literature. Large-vessel involvement in SLE is associated with high disease activity, and therefore, early recognition of this rare entity is essential.
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Conflicts of interest
There are no conflicts of interest.
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