There is no data on epidemiology of vasculitis from India. Vasculitic disorders are not common but are certainly under- diagnosed and under-reported. An idea of ...
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Vasculitis - Indian Perspective VR Joshi, Gayatri Mittal
Introduction There is no major systematic review on vasculitis from India. In this article we have relied mainly on data obtained from leading rheumatologists of the country. Reference to few other important publications has been made. To that extend the information is skewed. It is hoped that this attempt will help generate further data.
Epidemiology There is no data on epidemiology of vasculitis from India. Vasculitic disorders are not common but are certainly underdiagnosed and under-reported. An idea of the pattern of vasculitic disorders may be discerned from hospital-based data. Primary vasculitic disorders account for less than 1% cases seen in a rheumatology clinic. Chandrasekaran and Samant1,2 reported an incidence of 0.38%, 0.44% respectively. In our own experience these disorders account for 0.79% cases. Table 1 shows data on pattern of individual vasculitic disorders as seen by rheumatologists. A total of 1064 cases have been included. The data is an approximation as vasculitic disorders are seen and managed by other specialists and internists. Thus diseases like aortoarteritis, small vessel vasculitides and Kawasaki’s disease may be under-represented. For comparison frequency distribution of vasculitis in the American College of Rheumatology Criteria study (1990) is shown in Table 2.3
Diagnostic Problems Systemic necrotizing vasculitic disorders – PAN, WG, MPA and CSS – are often confused with infections notably tuberculosis.4 Pulmonary involvement with haemoptysis and radiologic appearances (justifiably) lead to suspicion of tuberculosis.4,5 While this is acceptable, it is pointed out that a diagnosis of vasculitis should be considered in any case of ‘unproven’ tuberculosis who fails to respond to standard therapy in 1-2 months. Further a day to day case of pulmonary tuberculosis does not have cutaneous, ocular, renal and peripheral nerve manifestations. Leucocytosis is another important clue. Precious time is lost and patients often present when significant organ damage is already present.4 Leprosy, viral infections and infective endocarditis are some other infections that cause diagnostic confusion. As a note of caution it must also be stated that vasculitis is often misdiagnosed for an infective disorder. Consultant Rheumatologist, PD Hinduja National Hospital & Research Centre, Veer Savarkar Marg, Mahim, Mumbai - 400 016.
Table 1: Frequency distribution of vasculitic disorders in India (N=1064)* Disease No. Percentage Aortoarteritis 215 20.20 Giant cell arteritis 35 3.36 Polyarteritis nodosa (PAN) 94 8.83 Cutaneous PAN 13 1.22 Wegener’s granulomatosis 147 13.81 Microscopic polyangiitis 42 3.94 Churg Straus syndrome 19 1.78 Henoch Schonlein purpura 232 21.80 Small vessel vasculitis 61 5.73 Behcet’s disease 145 13.62 Kawasaki disease 05 0.46 Undiagnosed 50 4.69 Others** 6 0.56 *Based on data from Bangalore, Baroda, Chandigarh, Chennai, Delhi, Hyderabad, Kolkata, Lucknow and Mumbai. **Cogan, Bazins, CNS vasculitis
Large Vessel Vasculitis Non-specific Aortoarteritis (Takayasu’s arteritis) Aortoarteritis has been well described and studied from India.6-9 Two monograms written by Indian authors are available on the subject.10,11 In contrast to Japanese patients in whom proximal aorta involvement is common (99%), in Indian patients descending and especially abdominal aorta involvement (reversed coarctation) is common (92%).6 Aortoarteritis is the commonest cause of renovascular hypertension in our context. 5 A high association with tuberculosis was described by Sen et al10 but not substantiated by others. Recently Aggarwala et al reported heightened immune response to Mycobacterium tuberculosis antigens, suggesting that this organism may have a role in the immunopathogenesis of this disease.13 Acute form is rarely diagnosed. Pulmonary involvement is reported as also involvement of coronary arteries. Sharma et al9 have reported persistent inflammatory activity for a prolonged period even when disease appears to be clinically silent. Mehra has reported an association with HLA B5 especially its alleles B51 and B52.8 Giant cell arteritis is distinctly uncommon in India. Kawasaki disease (KD) which affects paediatric age group has been reported from Chennai and Chandigarh,14,15 Both workers feel that KD is underdiagnosed in India.
Systemic Necrotising Vasculitides A. Wegener’s Granulomatosis (WG)
WG is being diagnosed more and more commonly.4,16,17 Clinical features and other data are shown in Table 3.
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Table 2: Frequency distribution of types of vasculitis in the American College of Rheumatology (1990) Criteria Study (N=959) Vasculitis
No
Percentage
Takayasu arteritis
63
6
Giant cell arteritis
214
22.3
Polyarteritis nodosa
118
12.3
Wegener’s granulomatosis
85
8.7
Henoch-Schonlein purpura
85
8.7
Hypersensitivity vasculitis
83
8.6
Kawasaki disease
52
5.4
Other vasculitis (types not specified)
129
Churg-Strauss syndrome
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Table 4: Summary of clinical features of PAN (N=40) M:F
4.5 : 1
Mean age (years) (range)
33.75 (6-72)
Duration of illness (months) (range)
6-9.5 months (3-10)
Constitution symptoms*
60% - 100%
Hypertension
58% - 100%
Renal**
6% - 50%
GI
14% - 57%
Mononeuritis multiplex
60% - 100%
CNS
14% - 29%
13.4
Skin
41% - 75%
2.1
Testicular pain
11.43%
Cardiac***
2.5%
Table 3: Clinical features of WG (N=60)4,16,17 Clinical features
Percentage (range)
Age (years) at diagnosis (range)
31.5 ± 41.5 (16-75)
M:F
1 : 14
Duration of symptoms (months) (range)
5.5 ± 8 (0.5 – 36)
*Includes musculoskeletal manifestations **May be less, % skewed by some smaller series ***Described in only one series.
Table 5: Sensitivity of diagnostic procedures in PAN
Constitutional symptoms
64-89
ENT
55-88
Lung
84-94
Eye
39-64
Kidney
65-73
Procedure
% positivity
Angiography
60-100
Nerve biopsy
50-100
Skin biopsy
50-75
Muscle biopsy*
50
Renal biopsy* * One study
Joints
44-59
Skin
18-39
CNS
4-28
Heart
4-12
C. Others
GIT
0-9
ANCA
70-80
This is based on data from Chandigarh and Delhi. Smaller number of cases are reported from other parts of India. This is partly due to under-reporting, but could well be a reflection of true geographical differences.
Most patients when diagnosed had systemic disease. Tuberculosis is the first diagnosis in 40%-50% cases. The radiological abnormalities include infiltrates (upto 100%) nodular shadows (upto 7%), cavities (upto 66%) and pleural effusion (upto 33%).5 Except Bambery’s series,4 where due to late diagnosis mortality was high (50%), others have reported good response to oral cyclophosphamide, prednisolone combination therapy, Most cases go into remission by six months. Relapses are frequent, but respond to second course of cyclophosphamide and prednisolone therapy.16 Contrary to Western literature side effects of CPM, are not as common.16 Long-term follow up has been unsatisfactory and information on long-term side effects like cancer etc. is not available. Data of 40 cases are depicted in Table 4. The disease manifestations do not differ from those reported in Western literature. The disease usually runs a monophasic course; chronic course is unusual. Table 5 shows the sensitivity of various diagnostic procedures (based on Indian data)
The number of reported cases of CSS and MPA is small and hence not analysed. Generally there do not seem any major differences from the Western data.
Others Behcet’s Disease There is only one large reported series from India.21 Bechet’s disease amongst Indians is a predominantly mucocutaneous disease. Articular, ocular and neurologic manifestations are uncommon. The age of onset is relatively late. Pathergy test is rarely +ve. No sex predominance is seen. Small Vessel Vasculitis HSP, HSV and others are common. Bagga et al22 have reported upon 47 cases of HSP. The systemic manifestations included abdominal pain (64%), nephritis (51% - 29% renal insufficiency), and arthralgias 47%. Crescentic glomerulonephritis carries a poor prognosis.
References 1.
Chandrasekaran AN, Venugopal B. Porkodi R, et al. Spectrum of clinical immunological features of systemic rheumatic disorders in a peripheral hospital in South India. J Indian Rheumat Assoc 1995;3:84-92.
2.
Samant R, Vaidya SS, Nadkar MY, Borges NE. Spectrum of clinical features of vasculitides in a referral hospital from Western India. J Indian Rheumat Assoc 1997;5:69-74.
3.
Bloch DA, Michel AB, Hunder GG et al. The American College of Rheumatology 1990 criteria for the classification of vasculitis: patients and methods. Arthritis Rheum 1990;33:1068-73.
B. Polyarteritis Nodosa (PAN)18-20
50
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Bambery P, Sakhuja V, Bhusnurmath SR, Jindal SK, Deodhar SD, Chugh KS. Wegener’s granulomatosis: clinical experience with eighteen cases. J Assoc Physicians India 1992;40:597-600.
13. Aggarwal A, Chag M, Sinha N, Naik S. Takayasu’s arteritis: role of Mycobacterium tuberculosis and its 65 Kda heat shock protein. International J Card 1996;55:49-55.
5.
Bambery P, Katariya S, Sakhuja V, et al. Wegener’s granulomatosis in North India. Radiologic manifestations in eleven patients. Acta Radiol 1988;29:11-13.
14. Narayanan SN, Krishnaveni K, Sabarinathan KD. Kawasaki Disease. Indian Pediatr 1997;34:139-43.
6.
Chugh KS, Jain S, Sakhuja V, et al. Renovascular hypertension due to Takayasu’s arteritis among Indian patients. J Med 1992;85:833-43.
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Sagar S, Ganguly NK, Koicha M, Sharma BK. Immunopathogenesis of TA. Heart Vessel (Suppl) 1992;7:85-90.
8.
Mehra NK, Jain R, Balamurugan A, et al. Immunogenetic analysis of Takayasu arteritis in Indian patients. International. J Cardiol 1998;66 (suppl. 1):27-32.
9.
Sharma BK, Jain S, Radotra BD. An autopsy study of Takayasu arteritis in India. International J Cardiol 1998;66 (suppl. 1):385-90.
10. Sen PK, Kinare SG, Kelkar MD, Parulkar GB. Non-specific aorto-arteritis - A monograph based on a study of 101 cases. Tata McGraw Hill Publication, Mumbai, 1971. 11. Panja M. Non-specific aorto-arteritis (Takayasu’s arteritis) - A monogram Publ. M. Panja, Kolkata 2001. 12. Yajima M, Numano F, Park YB, Sagar S. Comparative studies of patients with Takayasu arteritis in Japan, Korea and India - Comparison of clinical manifestations. Jpn Circ J 1994;58:914.
15. Singh S, Kumar L, Trehan A. Marwaha RK. Kawasaki disease at Chandigarh. Indian Pediatr 1997;34:822-25. 16. Kumar A, Pandhi A, Menon A, Sharma SK, Pande JN, Malaviya AN. Wegener’s granulomatosis in india: Clinical features, treatment and outcome of 25 patients. Indian J Chest Dis Allied Sci (under publication). 17. Handa R, Wali JP, Biswas A, Aggarwal P, Wig N. Wegener’s granulomatosis - a clinicopathologic study. J Assoc Physicians India 1997;45:536-39. 18. Gupta R, Kumar A, Malaviya AN. Outcome of polyarteritis nodosa in Northern India. J Rheumatol International 1997;17:101‑3. 19. Handa R, Wali JP, Gupta SD, et al. Classic polyarteritis nodosa and microscopic polyangiitis - a clinicopathologic study. J Assoc Physicians India 2001;49:314-319. 20. Kumar A, Malaviya AN, Bhat A, et al. Clinicopathological profile of vasculitides in India. J Assoc Physicians India 1985;33:694‑98. 21. Pande I, Uppal SS, Kailash S, Kumar A, Malaviya AN. Behcet’s disease in India: a clinical immunological, immunogenetic and outcome study. Br J Rheumatol 1995;34:825-30. 22. Bagga A, Kabra SK, Shrivastava RN, Bhuyan UN. Henoch Schonlein syndrome in northern Indian children. Indian Pediatr 1991;28:1153‑7.
