Rheumatology Advance Access originally published online on June 24, 2008
Rheumatology 2008 47(8):1257-1259; doi:10.1093/rheumatology/ken209
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Inflammatory aortitis controlled by the Chinese herbal remedy Donglingcao Pian
1Brighton and Sussex University Hospitals NHS Trust, Haywards Heath and 2Doncaster and Bassetlaw NHS Foundation Trust, Doncaster, UK
Correspondence to: K. M. Jordan, The Princess Royal Hospital, Lewes Road, Haywards Heath RH16 4EX, UK. E-mail: kelsey.jordan{at}bsuh.nhs.uk
SIR, GCA is a systemic vasculitis common in elderly patients. It most commonly affects extracranial branches of the carotid arteries such as the temporal arteries but also less commonly large vessels such as aorta and medium vessels such as coronary arteries.
In many cases, the diagnosis of GCA is straightforward. Patients typically present with unilateral headache, and have temporal artery tenderness on examination. The investigations show elevated inflammatory markers and biopsies of the temporal arteries may show typical inflammatory changes. Some patients may have classical PMR symptoms as there is an overlap between these two conditions [1]. A small proportion of patients can have clinically ‘silent’ GCA and may present as pyrexia of unknown origin or other systemic features such as weight loss or night sweats. In such cases, the diagnosis can be delayed.
Our patient had been well until 1992 when at the age of 54, following a viral illness, she developed chronic fatigue syndrome. Her symptoms changed in 2002 with fatigue, chest discomfort, dyspnoea and a feeling of pressure in her head but no temporal headache. She had lost 2 stone in weight over a few months, and complained of intermittent fevers and night sweats.
She was admitted for investigations at this time. General examination was unremarkable. In particular, all peripheral pulses were palpable and equal. There were no bruits detected. She had documented spikes in temperature. Investigations revealed a normocytic, normochromic anaemia of 10.1 g/dl, an elevated ESR at 102 mm/h and a CRP of 102 mg/l. Renal, liver and thyroid function tests were normal as were all tumour markers, ANA, ANCA and a serological screen (including hepatitis, HIV, TB and Legionella).
A chest X-ray, CT of her abdomen and pelvis, and a bone marrow biopsy were also normal. A PET scan showed increased uptake in the thoracic aorta and the great vessels suggestive of large-vessel vasculitis (Fig. 1a) (2003).
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She was referred to the rheumatology department with a diagnosis of GCA, and was treated with pulsed intravenous methylprednisolone followed by high-dose oral steroids that were gradually reduced. All blood indices normalized and she felt well.
By spring 2005 she was on oral prednisolone 2.5 mg daily. It was noted that her inflammatory markers were rising with each step reduction in prednisolone to a peak CRP of 12 over the preceding 6 months with no change in clinical features. A repeat PET scan confirmed ongoing inflammation in the thoracic aorta. She was advised to increase her prednisolone and plans were considered for the addition of AZA. At her next appointment she had failed to increase her prednisolone but had commenced Donglingcao Pian under the care of a professor in Chinese herbal medicine. She felt well and her CRP had normalized.
A repeat PET scan was performed to assess for ongoing inflammatory activity due to concern regarding her discontinued prednisolone (Fig. 1b) (2006). This did not show any evidence of active inflammation. Her ESR and CRP have remained normal ever since.
In GCA, the systemic inflammatory response is triggered by pro-inflammatory cytokines such as anti-TNF-
, IL-1 and IL-6. The histology of temporal artery biopsies in these patients demonstrates infiltration of activated macrophages and immunohistochemistry has shown excess TNF- [2, 3], suggesting a role for anti-TNF- drugs in the treatment of GCA. Matsuki et al. [4] have shown that IL-1 receptor antagonist-deficient mice develop aortitis, and this is dependent on TNF-. Hoffman et al. [5] showed no benefit in using infliximab with newly diagnosed GCA; however, this trial was small and only one dose of infliximab was evaluated. We must obviously consider the possibility that this patient may have gone into spontaneous remission.
Corticosteroids are first-line treatment for GCA. Immunosuppressants such as AZA and MTX are used as second-line agents. There have been case reports of patients with GCA who were resistant to conventional therapy but responded to anti-TNF- drugs such as infliximab [6] and etanercept [7].
Donglingcao Pian (Rabdosia rubescens) is a traditional herb in Chinese medicine used to treat inflammation. In China, it is also used to assist anti-cancer treatment (oesophagus, breast and liver) [8, 9]. It has also been found to have anti- TNF- properties, blocking TNF--stimulated NF-
B activity in human T cells [10]. Ikezoe et al. [10] also found that oridonin which is a diterpenoid purified from R. rubescens inhibited growth of multiple myeloma, acute lymphoblastic T-cell leukaemia and adult T-cell leukaemia cells in vitro.
Our patient received her Chinese medicine from an expert in traditional Chinese medicine. However, these agents can be ordered via the internet directly from the companies producing them. The herbal remedy used by this patient was not analysed to see if any other substances had been added to it, for example, steroids.
In conclusion, we describe a patient with an inflammatory aortitis controlled with a Chinese herbal remedy with possible anti-TNF- properties. There may be many potential new drugs to be found by investigating traditional herbal remedies that have been used for hundreds of years. However, unregulated use of some herbal remedies also has the potential of causing harm and even death.
Disclosure statement: The authors have declared no conflicts of interest.
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