• Users Online: 2307
  • Print this page
  • Email this page

  Table of Contents  
Year : 2012  |  Volume : 3  |  Issue : 1  |  Page : 66-68  

Aortoarteritis with systemic lupus erythematosus and secondary antiphopholipid syndrome

1 Department of Gen Medicine, NRS Medical College and Hospital, Kolkata, India
2 Department of Pharmacology, KPC Medical College and Hospital, Kolkata, India

Date of Web Publication3-Mar-2012

Correspondence Address:
Nandini Chakrabarti
358, N.S.C. Bose Road, Kolkata - 700 047
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2229-5178.93483

Rights and Permissions

How to cite this article:
Chakrabarti N, Chattopadhyay C. Aortoarteritis with systemic lupus erythematosus and secondary antiphopholipid syndrome. Indian Dermatol Online J 2012;3:66-8

How to cite this URL:
Chakrabarti N, Chattopadhyay C. Aortoarteritis with systemic lupus erythematosus and secondary antiphopholipid syndrome. Indian Dermatol Online J [serial online] 2012 [cited 2022 Jan 25];3:66-8. Available from: https://www.idoj.in/text.asp?2012/3/1/66/93483


A 39-year- old female patient presented to us with arthritis of small and large joints along with generalized eruptions all over her body [Figure 1] for the past 2 months. She had history of Raynaud's phenomenon and claudication of both upper and lower limbs for the past 15 years. In 1999, she had undergone an amputation of left forefoot due to gangrene. At that time she was diagnosed as a case of non-specific aortoarteritis. In 1990, she had been treated for pulmonary tuberculosis (TB). She suffered from three successive spontaneous mid-trimester abortions within last 3 years. She has no live offspring at present. There was no history of diabetes, hypertension, seizure, swelling of body, chest pain, or abdominal pain. She had received steroids irregularly in the past outside. Examination revealed an average built with mild pallor but no jaundice, edema, or lymphadenopathy. Pulses of left upper limb and popliteal, posterior tibialis, and dorsalis pedis of left lower limb were impalpable. There was no arterial tenderness or bruit. Blood pressure was 130/80 mm Hg in right upper limb; in left-sided limbs it could not be recorded. Skin involvement revealed lesions of a mixed character-some papulosquamous, others annular. There were also oral ulcers and malar rash. Ophthalmic and ENT evaluations were non-contributory. Cardio-respiratory, gastrointestinal, and neurological examinations were normal except slowed mentation. Investigations revealed Hb 11 g, %TC 9000, P 65%, L 30%, E 3%, B 2%, platelet count normal, and erythrocyte sedimentation rate 80 mm/h. Urine RE/ME was normal, fasting sugar 98 mg/dl, and creatinine 0.9 mg/dl. Chest X-ray revealed right upper lobe calcification. Sputum was negative. Mantoux test was positive (12 mm induration). HIV serology was negative. Antinuclear antibody (ANA) was positive at a titer of 1:320 by Hep 2 method, while Double stranded DNA (DSDNA) was negative. Considering the presence of malar rash, oral ulcer, arthritis of more than two joints, and this ANA titer, the diagnosis of systemic lupus erythematosus (SLE) was established (four American Rheumatological association (ARA) criteria fulfilled). History of recurrent fetal losses and gangrene of toes prompted us to go for anticardiolipin antibody. The results were IgM 9.5 μ/ml IgG 62 μ/ml (normal IgM < 5 unit/ml and IgG < 16 unit/ml). It was repeated after 6 weeks when values came out to be 10 and 70 μ/ml, respectively. Prothrombin time (PT) and apTT (Activated Partial thromboplastin time) were also prolonged. Presence of two clinical events (fetal loss and thrombosis) and positive anticardiolipin antibodies on two occasions point to a concomitant secondary antiphospholipid syndrome (APLA).
Figure 1: Photograph showing papulosquamous skin lesion

Click here to view

Doppler ultrasonography of peripheral vessels showed diastolic dampening of left brachial artery. Magnetic resonance (MR) angiogram of brain showed bilateral lacunar infarcts. MR angiography of lower limbs revealed narrowing of left femoral artery from mid-thigh extending along popliteal and anterior tibial arteries [Figure 2] and Computed tomography angiogram of aortic arch and its branches failed to visualize left subclavian artery beyond the first part [Figure 3]. Biopsy was taken from left temporal artery that showed accumulation of lymphocytes and few plasma cells in the tunica media with increased vascularity. There was some intimal proliferation as well. A diagnosis of nonspecific aortoarteritis and SLE, and secondary APLA was made. She was given local and oral steroids, hydroxychloroquine, and anticoagulants. Her skin lesions and arthritis gradually subsided.
Figure 2: MR angiogram of lower limbs-narrowing of left femoral artery extending down left popliteal and anterior tib

Click here to view
Figure 3: Computed tomography angiogram of aortic arch and main branches-left subclavian artery not visualized beyond first part

Click here to view

Aortoarteritis is more prevalent in Japan and Afro-Asian countries. Autopsy incidence in Japan is quoted as 33%. [1] It rarely coexists with SLE. Lupi herrara found ANA in 6% cases of Takayasu arteritis and LE cells in 2% cases. [2] Saxe and Altman reviewed 18 cases of non-specific aortoarteritis with SLE. Patients of both diseases have similar age of onset and female preponderance but presence of anticardiolipin antibody along with this is unique. [3] Most of the cases reported in literature had aortic aneurysms or dissections detected at autopsy or during surgery for dissections. [4]

In our case, five of six ACR criteria for classification of nonspecific aortoarteritis or Takayasu arteritis have been satisfied including age, h/o claudication, asynchronus pulses, and blood pressure and MR angiographic findings. SLE with secondary APLA was established on the basis of skin lesions, arthritis, and antibodies. The appearance of rash, arthritis, and abortion was preceded by claudication and gangrene by more than a decade. Possibly antibody work-up for APLA was not performed at that time. However, histopathology done later revealed features suggestive of large vessel vasculitis instead of the classical noninflammatory bland thrombosis so characteristic of APLA vasculopathy. Usually, SLE involves small and peripheral vessels but here involvement spans the whole gamut from small to medium to large vessels. This makes it a rare case report.

Another fascinating point to be noted is the occurrence of pulmonary TB in this patient. Pantell and Goodman had reviewed literature from 1961 to 1981 and found tuberculin test to be positive in 73.3-100% cases and active TB in 0.26-4.2%. [3] However, a large case series from central India has documented mantoux positivity in only 3.03% cases of aortoarteritis. [4] Several researchers from Western and Asian countries have attributed the association as coincidental. [5] A state of immunosuppression due to steroid therapy in our patient could have been the predisposing factor. The natural history of non-specific aortoarteritis is variable. But it usually progresses slowly with the development of hypertension, retinopathy, and aneurysm formation. The coexistence of the above with secondary APLA creates a hypercoagulable milieu that adversely affects the prognosis. [6] Furthermore, as the therapeutic strategies are radically different, recognition of the coexistence of antiphospholipid syndrome and aortoarteritis is of prime importance. [7]

   References Top

1.Deshpande J. Nonspecific aortoarteritis. J Postgrad Med 2000;46:1-2.  Back to cited text no. 1
[PUBMED]  Medknow Journal  
2.Lupi-Herrera E, Sánchez-Torres G, Marcushamer J, Mispireta J, Horwitz S, Vela JE. Takayasu's arteritis. Clinical study of 107 cases. Am Heart J 1977;93:94-103.  Back to cited text no. 2
3.Saxe PA, Altman RD. Takayasu's arteritis syndrome associated with systemic lupus erythematosus. Semin Arthritis Rheum 1992;5:295-305.  Back to cited text no. 3
4.Salkar RG, Parate R, Taori KB, Parate TR, Salkar HR, Mahajan S. Aortoarteritis: A study of 33 central Indian patients. Vascular 2003;13:61-6.  Back to cited text no. 4
5.Silver AS, Shao CY, Ginzler EM. Aortitis and aortic thrombus in systemic lupus erythematosus. Lupus 2006;15:541-3.  Back to cited text no. 5
6.Pantell RH, Goodman BW Jr. Takayasu's arteritis: The relationship with tuberculosis. Paediatrics 1981;67:84-8.  Back to cited text no. 6
7.Gupta A, Chandra N, Kler TS. Aortoarteritis with Systemic lupus erythematosus and secondary antiphospholipid antibody syndrome: A rare association. Indian Heart J 2002;54:301-3.  Back to cited text no. 7


  [Figure 1], [Figure 2], [Figure 3]

This article has been cited by
1 Gangrene in Takayasu’s arteritis: a report of two cases and review of literature
Durga Prasanna Misra,Abhra Chandra Chowdhury,Hira Lal,Namita Mohindra,Vikas Agarwal
Rheumatology International. 2016; 36(3): 449
[Pubmed] | [DOI]
2 Takayasu’s Arteritis with Systemic Lupus Erythematosus: A Rare Association
Dhrubajyoti Bandyopadhyay,Vijayan Ganesan,Debarati Bhar,Diptak Bhowmick,Sibnarayan Sasmal,Cankatika Choudhury,Sabyasachi Mukhopadhyay,Adrija Hajra,Manas Layek,Partha Sarathi Karmakar
Case Reports in Rheumatology. 2015; 2015: 1
[Pubmed] | [DOI]


    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

  In this article
    Article Figures

 Article Access Statistics
    PDF Downloaded320    
    Comments [Add]    
    Cited by others 2    

Recommend this journal