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 Table of Contents  
Year : 2019  |  Volume : 14  |  Issue : 4  |  Page : 304-311

Sustained remission in large-Vessel vasculitis: Do they ever burn out?

1 Department of Clinical Immunology and Rheumatology, Kalinga Institute of Medical Sciences, KIIT University, Bhubaneswar, Odisha, India
2 Centre for Arthritis and Rheumatism Excellence, Cochin, Kerala, India
3 Department of Clinical Immunology and Rheumatology, Christian Medical College, Vellore, Tamil Nadu, India
4 Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Date of Web Publication31-Dec-2019

Correspondence Address:
Prof. Ramnath Misra
Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow - 226 014, Uttar Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/injr.injr_182_19

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Drug-free remission is the ideal end point for any chronic disease. Although there are data on drug-free remission in rheumatoid arthritis and lupus, such data are limited for most vasculitis. Notably, there is less evidence for disease-modifying agents in large-vessel vasculitis (LVV). Thus, we explored the literature about sustained remission in LVV. MEDLINE and SCOPUS were searched for outcomes in LVV, and the results were manually screened for studies with at least 1-year follow-up. Isolated polymyalgia rheumatica was not included. In giant cell arteritis (GCA), histological and clinical remissions were discordant. Histology could not predict relapse rate. Various imaging techniques exhibit vessel wall inflammation in clinically quiescent disease. Relapse rate seems to correlate with the rate of steroid reduction. Relapse was rare when on higher steroid doses. Emerging evidence suggests that tocilizumab and methotrexate may prevent relapse. In Takayasu arteritis (TA), histology specimens are difficult to obtain. Remission on imaging does not mirror clinical remission. While magnetic resonance imaging and positron emission tomography are sensitive tools, these cannot differentiate smouldering disease from vascular repair. The best predictor of relapse is the extent of disease. Approximately half of the TA patients relapsed by 5 years. In patients undergoing intravascular procedures, restenosis occurred in around a third. Even for patients on anti- Tumour necrosis factor necrosis factor, sustained remission was in 20% only. LVV seems to be steroid dependent, and the efficacy of various steroid-sparing agents cannot be established unless the natural history of the disease is known. Both TA and GCA can have grumbling courses with relapse rates increasing over time.

Keywords: Drug tapering, drug-free remission, follow-up, giant cell arteritis, remission, Takayasu arteritis

How to cite this article:
Ahmed S, Shenoy P, Danda D, Misra R. Sustained remission in large-Vessel vasculitis: Do they ever burn out?. Indian J Rheumatol 2019;14:304-11

How to cite this URL:
Ahmed S, Shenoy P, Danda D, Misra R. Sustained remission in large-Vessel vasculitis: Do they ever burn out?. Indian J Rheumatol [serial online] 2019 [cited 2022 Jan 20];14:304-11. Available from:

  Introduction Top

Large-vessel vasculitis (LVV) predominantly affects large arteries. Arteries are considered to be “large” from the branches of the aorta to just before entering any viscera (or forming plantar or palmar arches).[1] Giant cell arteritis (GCA) and Takayasu arteritis (TA) are the two LVV. Both have overlapping and yet distinguishing features. Both can lead to significant morbidity and mortality.

TA usually occurs in young adults or children below 40 years of age, whereas GCA affects those above 60 years. Geographic distribution of both diseases does not seem to overlap. Data on GCA originate mostly from Europe and America, whereas data on TA are mostly from people with Asian or Latin American origins. GCA has a predilection for branches of the carotid arteries, whereas TA most commonly affects branches of the aorta. Histologically, there is no difference between the two.[2] While both seem to respond to glucocorticoids (GC), evidence for the efficacy of non-GC immunosuppressants is limited. Their genetics, pathology, and treatment responses are different.[3]

There have been randomized controlled trials (RCTs) of tumor necrosis factor (TNF)-α inhibitors in GCA that did not meet primary end points.[4],[5],[6] Two non-GC drugs have been demonstrated by RCTs to reduce relapse in GCA: interleukin-6 receptor antagonist tocilizumab and CTLA-4 Ig construct abatacept.[7],[8] Beyond GC, all other immunosuppressives seem to induce remission in atmost two-thirds of cases of TA.[9] RCTs are even less in TA, except for the two recently published.[10],[11] Given the limited evidence and the long-drawn-out course of these two chronic diseases, the natural history of both must be known. Besides, a significant issue for the clinician is when to stop treatment as immune-suppressant drugs have their risks.

Knowing the proportion of patients remaining in sustained remission with treatment can go a long way while designing valid clinical trials. Such knowledge can help the clinician determine when immunosuppressants can be tapered and stopped.

The ultimate goal for most rheumatic diseases is sustained drug-free remission. In rheumatoid arthritis, such remission has been reported from 3.6% to 22%.[12] There are hints that the application of the treat-to-target paradigm has gradually improved this incidence.[13] For systemic lupus erythematosus, such remission is uncommon. In lupus nephritis, it has been shown that relapse may occur even after being in remission for 15 years.[14]

In the case of vasculitis, data are comparatively limited. In antineutrophil cytoplasmic antibody-associated vasculitis, the combined follow-up data from various clinical trials seem to suggest that 30%–50% of patients relapse by 5 years.[15],[16] For polyarteritis nodosa (PAN), relapse rates at 5.5 years in a cohort of 348 patients were lower at around 20%, but mortality was also approximately 25%.[17] In a comparison between adult and childhood PAN, relapse rates were doubled for childhood systemic PAN (19.7/100 patient-years) than for adult systemic PAN (7.7/100 patient-years).[18] Higher disease activity at diagnosis predicts higher rates of relapse.[19]

Similar outcome data are scarce for LVV. With this in mind, we sought to review the available literature for outcomes in GCA and TA. This knowledge will help both in the design and interpretation of clinical trials and also help the clinician to plan and taper immunosuppressants for these two LVV.

  Search Strategy Top

A search was made using the PUBMED search engine with the string “((“Takayasu Arteritis” [MeSH] OR “Giant cell arteritis” [MeSH]) AND (outcome [ti] OR “recurrence” [MeSH] OR “natural history” [MeSH] OR “prognosis” [MeSH])) NOT (“case reports” [Publication Type])” as per recommendations on writing a biomedical review.[20] A similar search was made on SCOPUS. Inclusion was limited to studies available in English. All longitudinal studies that had a follow-up of at least 1 year with at least twenty patients of LVV were selected by manual screening. Preference was given to articles published in the last 10 years. The bibliographies of the selected studies were searched, and further relevant studies were also included. Studies relating only to polymyalgia rheumatica were not included.

  Giant Cell Arteritis Top

Tissue histopathology to predict relapse in giant cell arteritis

Recent studies have shown a low sensitivity of histopathology (~39%) in the diagnosis of GCA.[21] Thus, a biopsy may not be the ideal tool even to monitor response/remission in GCA. Therefore, it is challenging to validate clinical remission in GCA. The role of a second temporal artery biopsy was evaluated in forty GCA patients. Despite reaching clinical remission, around half had evidence of active vasculitis on the biopsy at the end of 1 year and granulomatous inflammation persisted.[22] This seems to be unequivocal evidence that inflammation still goes on in GCA even after “clinical” remission. Then again, the significance of this inflammation has not fully been understood. In a large study involving 343 temporal artery biopsies, 56 were documented to have only perivascular inflammation and no damage to the vessel intima or media. The outcomes of such 39 patients were no different from 39 matched controls at 5-year follow-up.[23] Thus, there is also a need for validated criteria for histological remission in GCA.

Imaging to predict relapses

The “Temporal Artery Biopsy-v-Ultrasound in diagnosis of Giant Cell Arteritis” study also showed a reduced sensitivity of ultrasound (~54%) in GCA diagnosis.[21] Ultrasound is not recommended as a standard follow-up for GCA, possibly as it is not sensitive enough. In a trial of thirty GCA patients, though tocilizumab led to complete clinical and laboratory remission, magnetic resonance angiography showed the persistence of inflammation in a third of cases.[24] Although the prognostic significance of this vessel-wall inflammation can be debated upon, it should not be overlooked until proven innocent beyond doubt! In a 10-year follow-up imaging study in Spain, it was shown that one-third of GCA patients develop aortic dilation or aneurysms despite continuing treatment.[25]

The use of positron emission tomography (PET) along with low-dose computed tomography has been recommended in the diagnosis of large-vessel vasculitides.[26] Studies have shown activity on PET in up to 58% of patients of GCA in apparent clinical remission.[27] It might be argued that PET is oversensitive. The same study demonstrated that patients with higher scores on PET Disease Activity Score have higher incidences of clinical relapses.

Relapses in giant cell arteritis

Relapses were frequent in the first 2 years from diagnosis when GCs were being tapered. Real-world data showed that in the presence of relapses, physicians would decrease GCs at half the speed. Patients with relapse took around 3 years to come down to 5-mg prednisolone (as compared to 1.7 years for nonrelapsing patients). The relapsing patients required a median of 6.5 years to be steroid free (compared to 3 years in the others).[28] [Table 1] summarizes time taken to reach low GC dose and corresponding relapse rates in different cohorts. It is noticeable that once-faster steroid withdrawal leads to more relapses. It again brings to mind the doubt about the usefulness of various conventional and biological steroid-sparing immunosuppressants that are being used today.
Table 1: Relapse rates in giant cell arteritis

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In GCA, there is an increased risk of dying due to large-vessel involvement (e.g., aortic aneurysm and hypertensive crisis), ischemic heart disease, or treatment-related condition (e.g., infection).[37] One study that looked at GCA with large-vessel (aorta and branches) involvement showed that the addition of a steroid-sparing immunosuppressant protected against cardiovascular events (heart rate [HR]: 0.44 [0.29–0.66], P < 0.0001) and dilatation of aorta (HR: 0.43 [0.23–0.77], P = 0.005). This study also showed that large-vessel involvement is an independent predictor for cardiovascular events.[38] A meta-analysis of all treatments for GCA demonstrated that tocilizumab and methotrexate significantly increased the relative risk of being relapse free.[39]

In addition, a subset of LVV has been described that had onset between 50 and 60 years of age. This group seems to have more of aorta involvement, peripheral limb vessel involvement, and more refractory disease than gender-matched LVV with onset after 60 years (GCA or isolated idiopathic aortitis).[40]

  Takayasu Arteritis Top

Histopathology in Takayasu arteritis

Histopathology is often less available for TA, but there are case reports that point out the discrepancies between clinical disease activity and inflammation on biopsies.[41] There are hypotheses that vascular and systemic inflammation in TA are distinct processes: the systemic inflammation is driven by interleukin-6 (IL-6)/Th17/IL-17 pathway, while the vascular inflammation depends on IL-12/Th1/interferon-gamma (IFN-γ) pathway.[42] Drugs such as tocilizumab might abrogate the systemic manifestations such as fever, malaise, and raised acute-phase reactants, but Th1/IFN-γ-driven vascular inflammation and damage might still progress unhindered. As biopsies of the aorta and its branches are not easy to obtain, histopathology cannot be used to predict relapses.

Imaging to predict relapses

In a study of 52 TA patients in Brazil, nine were classified as having monophasic illness on follow-up (median 74.3 months). However, imaging showed that three out of the nine patients in apparent remission also developed new arterial lesions on follow-up.[43] Thus, clinical remission is harder to define in TA as compared to GCA. Outcome Measure in Rheumatology (OMERACT) has drafted a core set of outcome measures of LVV.[44] One of these is arterial function and is expected to include imaging.

In a study from Korea, 59 apparently “inactive” TA at diagnosis were followed up. Of these, 13 (22%) had a clinical relapse, and the best predictor of relapse was the extent of disease and Numano type 5 involvement. Around two-thirds of patients clinically labeled as inactive showed evidence of vessel wall inflammation on magnetic resonance imaging.[45] As in the case of GCA, PET may show vascular uptake even in clinical remission. It is not known how to distinguish smothering inflammation from vascular repair.[46] Thus, there is a mismatch between clinical assessment and imaging in assessing disease activity in TA. Long-term follow-up studies are required to recognize surrogates for disease activity on imaging and then validate these.

Relapses in Takayasu arteritis

There is discrepancy between levels of acute-phase reactants and vasculitis activity in TA.[47] Thus, clinical definitions of remission are far from foolproof, and a definite proportion of patients will progress silently without any systemic symptoms. Predictably, patients with higher disease burden at baseline have higher chances of relapse. [Table 2] summarizes studies on Takayasu relapses.
Table 2: Relapse in Takayasu arteritis

Click here to view

From [Table 2], it can be surmised that TA seems to GC responsive, but relapse rates increase as the duration of follow-up increases. There is little data on the efficacy of the different steroid-sparing agents in TA. However, randomized controlled trials of biologicals in TA have been underpowered.[9]

Follow-up studies of childhood TA are less: One report has shown 9% (5/55 patients) mortality at a mean 3.2-year follow-up. Three patients had nephrectomies, two thoracic aortic stentings, and five had angioplasties.[55] No data is provided about relapse, but the study shows the abysmal prognosis of childhood TA.

Postinterventional outcomes in Takayasu arteritis

There are limited series that have reported long-term follow-up after various vascular intervention in TA. The initial studies suggested that interventions are best done at a time when the disease is quiescent and after initiation of immunosuppression.[56] The summary of outcomes in recent literature is available in [Table 3]. Restenosis occurred in around one-third of the patients undergoing endovascular procedures. Although open surgeries including grafting show less restenosis, these are often technically demanding and are being performed only in a limited number of specialized centers.
Table 3: Outcomes after vascular surgery or endovascular interventions in Takayasu arteritis

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For children, data are even sparse regarding interventions. One study looked at ten children with TA, of which 8 had surgical procedures, one balloon angioplasty, and one had both. In 20-year follow-up, one had sudden death, while the progression of TA was detected in two.[62] As these were severe cases, possibly children might be having a slightly better outcome with interventions than adults in terms of progression. In another study, eight children had undergone interventions, of whom two failed angioplasty and had to undergo renal artery bypass.[63]

Follow-up of anti-tumor necrosis factors in Takayasu arteritis

The effect of drug therapy on TA is beyond the scope of this review. However, two studies have reported long-term follow-up of patients on anti-TNF-α. The first report from Minnesota had 20 patients (17 infliximab, 2 adalimumab, and 1 etanercept) followed up for 23 months: 33% had a relapse while on anti-TNFs and 20% discontinued the treatment due to adverse events.[64] The second included 97 patients from Norway: even those diagnosed after 2000 (“late cohort”) developed a median of one new lesion at the last follow-up and 44% had sustained remission up to 5 years.[65] It is noteworthy that sustained remission in the conventional DMARD group of this study was merely 20%. Thus, it is difficult to be sure if this was the effect of the DMARDs or just the natural course of the disease!!!

  Conclusion Top

Both GCA and TA can have a prolonged grumbling course. Often, clinically quiescent disease can present with new lesions on angiography. In both conditions, relapses usually occur on tapering steroids. For GCA, there is emerging evidence for tocilizumab and methotrexate (and possibly leflunomide) as steroid-sparing agents. In the case of TA, the evidence is less for non-GC drugs.

In GCA, relapse rates are coming down as patients are being tapered more slowly off GCs. The latest data show that around one-third relapse by 2–3 years. For TA, roughly one-third remain in remission on drugs by the end of 5 years, with the relapse rate increasing with longer follow-ups. Thus, until we understand these diseases better, we should be cautious with drug tapering. Even if drugs are stopped, patients should be monitored lifelong.

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Conflicts of interest

There are no conflicts of interest.

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  [Table 1], [Table 2], [Table 3]


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