LETTERS TO THE EDITOR
P.S. HELLIWELL, V. WRIGHT
School of Medicine, Rheumatology and Rehabilitation Research Unit, The University of Leeds, Clarendon Road, Leeds LS2 9N2. Accepted 21 February 1992 1. Arnett FC, Edworthy SM, Bloch DA et al. The American Rheumatism Association, 1987, revised criteria for the classification of rheumatoid arthritis. Arthritis Rheum 1988;31:315-24. 2. Moll JMH, Wright V. Psoriatic arthritis. Semin Arthritis Rheum 1973;3:55-78.
Polymyalgia Rheumatica and Panarteritis Nodosa SIR—We read with interest the report of Herrero Beaumont et al. [1]. We do agree with them that polymyalgia rheumatica (PMR) is a symptom complex with different disease associations, being most strongly associated with giant cell arteritis (GCA). However, other vasculitides are also associated. We present here a patient with panarteritis nodosa (PAN) mimicking PMR and temporal arteritis. A 72-year-old female presented with upper and lower limb girdle pain, jaw claudication, sudden development of headache and constitutional symptoms. Physical examination revealed a normal temporal pulse and pain on active movements on both shoulders. General examination was normal. Investigations revealed a sedimentation rate of 65, Hb 11.1 g/dl, WBC 12.6 x 107l, platelets 315 x 107l. ANA antibodies and rheumatoid factor were negative. ANCA antibodies were positive with a perinuclear immunofluorescence pattern (pANCA). Renal function and urine analysis were normal. A chest X-ray was normal. Temporal artery biopsy showed a normal main artery though a small adjacent artery showed necrotizing vasculitis. Treatment was begun with oral steroids 50 mg/day and symptoms improved. Two months later symptoms recurred. Investigations revealed a deterioration of renal function and microhaematuria. Kidney biopsy showed fibrinoid necrosis in small vessels and arterioles and a focal and segmental necrotizing glomerulonephritis. Immunofluorescence studies demonstrated no immunoglobulins in the renal tissue. Treatment was begun with cyclophosphamide 100 mg/day with a subsequent resol-
ution of symptoms. Eighteen months later the patient was asymptomatic with normal renal function and acute phase reactants. She is now off steroids and taking oral cyclophosphamide 50 mg/day. PMR with or without temporal arteritis can masquerade as panarteritis nodosa [2]. The term temporal arteritis includes different types of vasculitis, GCA being one of them [3]. Morgan [4] described two cases of polyarteritis and one of hypersensitivity vasculitis diagnosed by temporal artery biopsy. Frayha [5] reported a case of PAN presenting as temporal arteritis. The term non-giant cell arteritis is reserved for this group of patients. Saveuse describes temporal arteritis as a syndrome which most frequently includes GCA and occasionally PAN [3]. Our patient presented to us with a syndrome of PMR and temporal arteritis. Temporal artery biopsy yielded necrotizing vasculitis in a small vessel even though the temporal artery was almost normal. Treatment with oral steroids was begun, and the patient recovered. Subsequently, renal function deteriorated and kidney biopsy showed typical changes of microscopic and macroscopic PAN. In summary, polymyalgia rheumatica may occur as an early feature of PAN, as well as in association with any systemic vasculitis [1,6]. E . CASTELLOTE,* I. OjANGUREN,t J- TEIXIDO,* A. SERRA,* A. OLIVER
* Service of Nephrology, tService of Pathology, tSectionof Rheumatology, Hospital Universitario 'Germans Trias i Pujol', Barcelona, Spain Accepted 17 February 1992 1. Herrero-Beaumont G, Armas JB, Amorim R, Shenstone B, Bhalla A, Maddison PJ. Limited forms of Wegener's granulomatosis presenting as polymyalgia rheumatica. Br J Rheumatol 1991;3O:382^4. 2. Siame JL. Pseudo-polyarthrite rhizom61ique et periartdnte noueuse. Presse Mid 1985; 14:427-6. 3. Saveuse A, Dorra M, Dechy H, Baglin C, Rouveix E, Betourne CL. L'art6rite temporale: un syndrome. Presse Mid 1988;17:517-20. 4. Morgan GJ, Harris ED, Non giant cell arteritis: three cases and review of the literature. Arthritis Rheum 1978;21:362-6. 5. Frayha RA, Abu-Haidar F. Polyarteritis nodosa masquerading as temporal arteritis. J Rheumatol 1979;6:76-9. 6. Conn DL. Update on systemic necrotizing vasculitis. Mayo Gin Proc 1989;64:535-43.
Steroid Treatment in Giant Cell Arteritis SIR—We were interested to read the recent paper by Miles et al. [1] in your journal, but we disagree with their conclusion that '20 mg prednisolone a day is an adequate starting dose for treatment of giant cell arteritis, providing a very high degree of protection from serious ocular complications'. The authors do not state the number of patients, if any, in their series who presented because of visual loss (as opposed to other symptoms), and consequently their conclusions should not be applied to this large and clinically important group. Overall it is thought that approximately 50% of patients presenting with giant cell artertis (GCA) do so because of visual loss [2]. About one third of this 50% progress to bilateral blindness if untreated, the second eye usually becoming involved within 10 days of the first [3]. Consequently the primary aim of steroid treatment in GCA is protection of the visual system. Eyes affected by GCA are often rendered blind to the extent of 'no perception of light' [4], so bilateral involvement is profoundly disabling.
Downloaded from http://rheumatology.oxfordjournals.org/ at UQ Library on June 19, 2015
Sir—We are grateful to Doctors Veale and FitzGerald for their comments. However, we feel that any clinical taxonomy must be flexible and responsive to new observations and we would defend our revised classification on the following grounds. Firstly, the patients in this study were not highly selected: the only criteria for selection were diagnosis of psoriatic arthritis and previous attendance at the rheumatology clinic. Secondly, our definition of joint symmetry is based on convention [1]; if symmetry depended on matching joint for joint then few cases of rheumatoid arthritis (never mind psoriatic arthritis) would meet this criterion. What we have done is to redefine subjects with psoriatic arthritis using similar groupings to those originally described by Moll and Wright [2] but with stricter (and explicitly stated) definitions. Thirdly, the finding of abnormal isotope uptake in manubrio-sternal and sterno-clavicular joints of many of our patients provided the link to a condition previously only described in association with a distinct variant of psoriasis palmo-plantar pustulosis. Our proposed classification serves to encompass this new information and provides the framework for further investigations concerning aetiopathogenesis and prognosis.
431
P.S. HELLIWELL, V. WRIGHT
School of Medicine, Rheumatology and Rehabilitation Research Unit, The University of Leeds, Clarendon Road, Leeds LS2 9N2. Accepted 21 February 1992 1. Arnett FC, Edworthy SM, Bloch DA et al. The American Rheumatism Association, 1987, revised criteria for the classification of rheumatoid arthritis. Arthritis Rheum 1988;31:315-24. 2. Moll JMH, Wright V. Psoriatic arthritis. Semin Arthritis Rheum 1973;3:55-78.
Polymyalgia Rheumatica and Panarteritis Nodosa SIR—We read with interest the report of Herrero Beaumont et al. [1]. We do agree with them that polymyalgia rheumatica (PMR) is a symptom complex with different disease associations, being most strongly associated with giant cell arteritis (GCA). However, other vasculitides are also associated. We present here a patient with panarteritis nodosa (PAN) mimicking PMR and temporal arteritis. A 72-year-old female presented with upper and lower limb girdle pain, jaw claudication, sudden development of headache and constitutional symptoms. Physical examination revealed a normal temporal pulse and pain on active movements on both shoulders. General examination was normal. Investigations revealed a sedimentation rate of 65, Hb 11.1 g/dl, WBC 12.6 x 107l, platelets 315 x 107l. ANA antibodies and rheumatoid factor were negative. ANCA antibodies were positive with a perinuclear immunofluorescence pattern (pANCA). Renal function and urine analysis were normal. A chest X-ray was normal. Temporal artery biopsy showed a normal main artery though a small adjacent artery showed necrotizing vasculitis. Treatment was begun with oral steroids 50 mg/day and symptoms improved. Two months later symptoms recurred. Investigations revealed a deterioration of renal function and microhaematuria. Kidney biopsy showed fibrinoid necrosis in small vessels and arterioles and a focal and segmental necrotizing glomerulonephritis. Immunofluorescence studies demonstrated no immunoglobulins in the renal tissue. Treatment was begun with cyclophosphamide 100 mg/day with a subsequent resol-
ution of symptoms. Eighteen months later the patient was asymptomatic with normal renal function and acute phase reactants. She is now off steroids and taking oral cyclophosphamide 50 mg/day. PMR with or without temporal arteritis can masquerade as panarteritis nodosa [2]. The term temporal arteritis includes different types of vasculitis, GCA being one of them [3]. Morgan [4] described two cases of polyarteritis and one of hypersensitivity vasculitis diagnosed by temporal artery biopsy. Frayha [5] reported a case of PAN presenting as temporal arteritis. The term non-giant cell arteritis is reserved for this group of patients. Saveuse describes temporal arteritis as a syndrome which most frequently includes GCA and occasionally PAN [3]. Our patient presented to us with a syndrome of PMR and temporal arteritis. Temporal artery biopsy yielded necrotizing vasculitis in a small vessel even though the temporal artery was almost normal. Treatment with oral steroids was begun, and the patient recovered. Subsequently, renal function deteriorated and kidney biopsy showed typical changes of microscopic and macroscopic PAN. In summary, polymyalgia rheumatica may occur as an early feature of PAN, as well as in association with any systemic vasculitis [1,6]. E . CASTELLOTE,* I. OjANGUREN,t J- TEIXIDO,* A. SERRA,* A. OLIVER
* Service of Nephrology, tService of Pathology, tSectionof Rheumatology, Hospital Universitario 'Germans Trias i Pujol', Barcelona, Spain Accepted 17 February 1992 1. Herrero-Beaumont G, Armas JB, Amorim R, Shenstone B, Bhalla A, Maddison PJ. Limited forms of Wegener's granulomatosis presenting as polymyalgia rheumatica. Br J Rheumatol 1991;3O:382^4. 2. Siame JL. Pseudo-polyarthrite rhizom61ique et periartdnte noueuse. Presse Mid 1985; 14:427-6. 3. Saveuse A, Dorra M, Dechy H, Baglin C, Rouveix E, Betourne CL. L'art6rite temporale: un syndrome. Presse Mid 1988;17:517-20. 4. Morgan GJ, Harris ED, Non giant cell arteritis: three cases and review of the literature. Arthritis Rheum 1978;21:362-6. 5. Frayha RA, Abu-Haidar F. Polyarteritis nodosa masquerading as temporal arteritis. J Rheumatol 1979;6:76-9. 6. Conn DL. Update on systemic necrotizing vasculitis. Mayo Gin Proc 1989;64:535-43.
Steroid Treatment in Giant Cell Arteritis SIR—We were interested to read the recent paper by Miles et al. [1] in your journal, but we disagree with their conclusion that '20 mg prednisolone a day is an adequate starting dose for treatment of giant cell arteritis, providing a very high degree of protection from serious ocular complications'. The authors do not state the number of patients, if any, in their series who presented because of visual loss (as opposed to other symptoms), and consequently their conclusions should not be applied to this large and clinically important group. Overall it is thought that approximately 50% of patients presenting with giant cell artertis (GCA) do so because of visual loss [2]. About one third of this 50% progress to bilateral blindness if untreated, the second eye usually becoming involved within 10 days of the first [3]. Consequently the primary aim of steroid treatment in GCA is protection of the visual system. Eyes affected by GCA are often rendered blind to the extent of 'no perception of light' [4], so bilateral involvement is profoundly disabling.
Downloaded from http://rheumatology.oxfordjournals.org/ at UQ Library on June 19, 2015
Sir—We are grateful to Doctors Veale and FitzGerald for their comments. However, we feel that any clinical taxonomy must be flexible and responsive to new observations and we would defend our revised classification on the following grounds. Firstly, the patients in this study were not highly selected: the only criteria for selection were diagnosis of psoriatic arthritis and previous attendance at the rheumatology clinic. Secondly, our definition of joint symmetry is based on convention [1]; if symmetry depended on matching joint for joint then few cases of rheumatoid arthritis (never mind psoriatic arthritis) would meet this criterion. What we have done is to redefine subjects with psoriatic arthritis using similar groupings to those originally described by Moll and Wright [2] but with stricter (and explicitly stated) definitions. Thirdly, the finding of abnormal isotope uptake in manubrio-sternal and sterno-clavicular joints of many of our patients provided the link to a condition previously only described in association with a distinct variant of psoriasis palmo-plantar pustulosis. Our proposed classification serves to encompass this new information and provides the framework for further investigations concerning aetiopathogenesis and prognosis.
431
