545
9
Section of Rheumatology & Rehabilitation
revealed a reduction in labile crosslinks, suggesting that D-penicillamine was inhibiting new dermal collagen formation. Despite an episode of exfoliative dermatitis she remained on D-penicillamine therapy with steroid cover and continued to improve. The patient was readmitted in June 1974 as an emergency due to the sudden onset of grand mal convulsions, cerebral cedema and renal failure. Her blood pressure was 170/120 mmHg, blood urea 170 mg/100 ml, and there was oliguria. Despite peritoneal dialysis she eventually died in pulmonary cedema. At post-mortem, apart from typical skin changes, the kidneys showed evidence of interlobular fibrosis in the parenchyma, arteriolar intimal fibrous proliferation and glomerular tuft swelling with fibrinoid necrosis. These changes confirmed the diagnosis of PSS with renal involvement.
REFERENCES Bailey A J, Peach C M & Fowler L J (1970) BiochemicalJournal 117,819 Bennett R, Bluestone R, Holt P J L & Bywatern E G L (1971) Annals of the Rheumatic Diseases 30, 581-588 Cannon P J, Hassar M, Case D B, Casarella W J, Sommers S C & LeRoy E C (1974) Medicine (Baltimore) 53, 1-46 Farmer R G, Gifford R W & Hines E A (1960) Circulation 21, 1088 Herbert C M, Lindberg K A, Jayson M I V & Bailey A J (1974) Lancet i, 187-192
Discussion
Spinal tuberculosis is not yet a disease of the past in Great Britain (Jackson 1971).
Renal involvement in PSS is of bad prognostic significance and is a frequent cause of death (Farmer et al. 1960, Bennett et al. 1971). Cannon et al. (1974) found that 45% of 210 patients with PSS had markers of renal involvement; 13 % had hypertension, proteinuria and azotemia and half of these had malignant hypertension, as in our patient. At follow up only 10% of those with renal markers were alive, compared to 60% of those without renal involvement. In particular, azotemia and malignant hypertension were most serious, with a mean survival of less than one month. Renal histology was similar to that in our patient. Some of the renal changes may be due to proliferation of interlobular and glomerular basement membrane collagen. Proliferation of intimal collagen could cause renal vascular changes, but equally such proliferation could be secondary to hypertension. In our patient there was clinical and biochemical evidence of reduction in new collagen formation in the skin during D-penicillamine therapy. This drug, however, did not prevent the development of rapidly advancing renal involvement and malignant hypertension. A possible explanation for this apparent paradox is the type of crosslinking found in the different forms of collagen in skin and kidney. In collagen there are two types of crosslinks, namely the aldimine and keto forms. In scleroderma skin it is the former type that proliferates, and this crosslink can be cleaved by D-penicillamine. Work is in progress to determine the type of crosslink in renal collagen in scleroderma, but it is possible that it is the keto form which is resistant to Dpenicillamine. For this reason the drug may be effective for cutaneous disease but not for the renal or other systemic manifestations.
Spinal Tuberculosis (Two Cases) N E Williams MA MRCP (for D A H Yates MD FRCP) (Department of Rheumatology, St Thomas' Hospital, London SE] 7EH)
Case 1 A 28-year-old man from Mauritius presented with a four-month history of low back pain and left-sided sciatica. Examination and investigations at that time did not reveal any abnormality apart from a raised ESR. He was seen regularly over the next four months, when he suddenly became pyrexial with a hot, red, fluctuant swelling over the left sacroiliac joint. Tomography of the sacrum showed no lesion of the sacroiliac joint but a destructive lesion of the body of SI and a large presacral abscess. The abscess was drained and the major part of the left side of the sacrum removed. Histology and culture confirmed the lesion to be tuberculous. With antituberculous therapy the patient made an excellent recovery.
This case illustrates well the lack of radiological change in early spinal tuberculosis. Case 2 A 53-year-old London woman presented with a six-month history of thoracic pain of increasing severity, later accompanied by root pain. Examination showed pain on thoracic rotation with tenderness over the spinous processes of T9 and T10. ESR was raised and X-ray of the thoracic spine showed a destructive lesion of the body of T9 with a paravertebral soft tissue mass. An abscess cavity was drained and subsequent culture showed the lesion to be tuberculous. Following three months' bed rest and antituberculous therapy the patient was slowly mobilized, with a recurrence of pain. X-ray showed further destruction, with a persistence of the paravertebral mass. Re-exploration with spinal
546 Proc. roy. Soc. Med. Volume 68 September 1975 fusion was considered necessary, and the patient was fitted into a plaster boat; she found this extremely stressful, and she suddenly collapsed and died. At post-mortem both adrenal glands were completely destroyed by fresh tuberculosis, in spite of the fact that the organism cultured in the laboratory was sensitive to all three antituberculous agents the patient had received. Another interesting point is that at no time until her collapse did her blood pressure fall below 110/80 and that on several occasions her electrolytes were normal. There is still no universal agreement on the treatment of spinal tuberculosis, and this led the Medical Research Council to set up a series of controlled trials; the first four reports have now been published (Medical Research Council 1973a, b, 1974a, b). These reports indicate that where facilities exist the 'Hong Kong' radical operation with chemotherapy gives the best result, but simple out-patient therapy with PAS and INAH may be relied on to give good results. Recent reports on the treatment of pulmonary tuberculosis with a combination of drugs including Rifampicin (East African/British Medical Research Councils 1973) for only six months gave very good results. Further study of this regime in the treatment of spinal tuberculosis is yet to be evaluated. REFERENCES East African/British Medical Research Councils (1973)Lanceti, 1331-1339 Jackson J W (1971) Postgraduate Medical Journal 47, 723-724 Medical Research Council (1973a) Journal of Bone and Joint Surgery 55B, 678 (1973b) Tubercle 54, 261 (1974a) Journal of Tropical Medicine and Hygiene 77, 72 (1974b) British Journal of Surgery 61, 853
Meeting 9 January 1974 The subject was Pain, and the speakers were Professor P D Wall (Cerebral Functions Group, Department of Anatomy and Embryology, University College London) and Dr Barry Wyke (Neurological Laboratory, Royal College of Surgeons, London). Meeting 8 May 1974 Dr D M L Doran (West Middlesex Hospital,
Isleworth) delivered his Presidential Address, entitled Is your Treatment really Necessary?
10 Meeting 28-29 June 1974 with the British Association for Rheumatology and Rehabilitation, the Swiss Societyfor Rheumatology, and the Heberden Society, at the University ofBristol
The following papers were read: Pain Threshold Analysis in Patients with Osteoarthrosis of the Hip Dr Susan L O'Driscoll (Department of Medicine, University ofBristol) and Dr Malcolm I V Jayson (Royal National Hospitalfor Rheumatic Diseases, Bath) REFERENCE O'Driscoll S & Jayson M I V (1974) British Medical Journal iii, 714-715
Lower Motor Neurone Weakness in Rheumatoid Cervical Myelopathy Dr J A Mathews (Department ofRheumatology, St Thomas' Hospital, London SEJ) REFERENCE Mathews J A (1975) Scandinavian Journal of Rheumatology 4, Suppl. 8, No. 064
Capillary Resistance and Back Pain Dr B J Sweetman and Dr J A D Anderson (Arthritis Research Unit, Guy's Hospital, London SEJ) REFERENCE Sweetman B J & Anderson J A D (1975) Rheumatology and Rehabilitation 14, 1
Meeting 12 February 1975 with the Society for Back Pain Research
The subject of the meeting was Criteria for the Diagnosis of Acute Intervertebral Disc Syndromes, and the principal speakers were Dr John A Mathews (St Thomas' Hospital, London SEI 7EH), Mr Martin Nelson (Leeds General Infirmary, Leeds 1) and Dr Marcia Wilkinson (Regional Neurological Unit, Eastern Hospital, London E9 6B Y). The meeting will be reported in Rheumatology & Rehabilitation, August 1975.
9
Section of Rheumatology & Rehabilitation
revealed a reduction in labile crosslinks, suggesting that D-penicillamine was inhibiting new dermal collagen formation. Despite an episode of exfoliative dermatitis she remained on D-penicillamine therapy with steroid cover and continued to improve. The patient was readmitted in June 1974 as an emergency due to the sudden onset of grand mal convulsions, cerebral cedema and renal failure. Her blood pressure was 170/120 mmHg, blood urea 170 mg/100 ml, and there was oliguria. Despite peritoneal dialysis she eventually died in pulmonary cedema. At post-mortem, apart from typical skin changes, the kidneys showed evidence of interlobular fibrosis in the parenchyma, arteriolar intimal fibrous proliferation and glomerular tuft swelling with fibrinoid necrosis. These changes confirmed the diagnosis of PSS with renal involvement.
REFERENCES Bailey A J, Peach C M & Fowler L J (1970) BiochemicalJournal 117,819 Bennett R, Bluestone R, Holt P J L & Bywatern E G L (1971) Annals of the Rheumatic Diseases 30, 581-588 Cannon P J, Hassar M, Case D B, Casarella W J, Sommers S C & LeRoy E C (1974) Medicine (Baltimore) 53, 1-46 Farmer R G, Gifford R W & Hines E A (1960) Circulation 21, 1088 Herbert C M, Lindberg K A, Jayson M I V & Bailey A J (1974) Lancet i, 187-192
Discussion
Spinal tuberculosis is not yet a disease of the past in Great Britain (Jackson 1971).
Renal involvement in PSS is of bad prognostic significance and is a frequent cause of death (Farmer et al. 1960, Bennett et al. 1971). Cannon et al. (1974) found that 45% of 210 patients with PSS had markers of renal involvement; 13 % had hypertension, proteinuria and azotemia and half of these had malignant hypertension, as in our patient. At follow up only 10% of those with renal markers were alive, compared to 60% of those without renal involvement. In particular, azotemia and malignant hypertension were most serious, with a mean survival of less than one month. Renal histology was similar to that in our patient. Some of the renal changes may be due to proliferation of interlobular and glomerular basement membrane collagen. Proliferation of intimal collagen could cause renal vascular changes, but equally such proliferation could be secondary to hypertension. In our patient there was clinical and biochemical evidence of reduction in new collagen formation in the skin during D-penicillamine therapy. This drug, however, did not prevent the development of rapidly advancing renal involvement and malignant hypertension. A possible explanation for this apparent paradox is the type of crosslinking found in the different forms of collagen in skin and kidney. In collagen there are two types of crosslinks, namely the aldimine and keto forms. In scleroderma skin it is the former type that proliferates, and this crosslink can be cleaved by D-penicillamine. Work is in progress to determine the type of crosslink in renal collagen in scleroderma, but it is possible that it is the keto form which is resistant to Dpenicillamine. For this reason the drug may be effective for cutaneous disease but not for the renal or other systemic manifestations.
Spinal Tuberculosis (Two Cases) N E Williams MA MRCP (for D A H Yates MD FRCP) (Department of Rheumatology, St Thomas' Hospital, London SE] 7EH)
Case 1 A 28-year-old man from Mauritius presented with a four-month history of low back pain and left-sided sciatica. Examination and investigations at that time did not reveal any abnormality apart from a raised ESR. He was seen regularly over the next four months, when he suddenly became pyrexial with a hot, red, fluctuant swelling over the left sacroiliac joint. Tomography of the sacrum showed no lesion of the sacroiliac joint but a destructive lesion of the body of SI and a large presacral abscess. The abscess was drained and the major part of the left side of the sacrum removed. Histology and culture confirmed the lesion to be tuberculous. With antituberculous therapy the patient made an excellent recovery.
This case illustrates well the lack of radiological change in early spinal tuberculosis. Case 2 A 53-year-old London woman presented with a six-month history of thoracic pain of increasing severity, later accompanied by root pain. Examination showed pain on thoracic rotation with tenderness over the spinous processes of T9 and T10. ESR was raised and X-ray of the thoracic spine showed a destructive lesion of the body of T9 with a paravertebral soft tissue mass. An abscess cavity was drained and subsequent culture showed the lesion to be tuberculous. Following three months' bed rest and antituberculous therapy the patient was slowly mobilized, with a recurrence of pain. X-ray showed further destruction, with a persistence of the paravertebral mass. Re-exploration with spinal
546 Proc. roy. Soc. Med. Volume 68 September 1975 fusion was considered necessary, and the patient was fitted into a plaster boat; she found this extremely stressful, and she suddenly collapsed and died. At post-mortem both adrenal glands were completely destroyed by fresh tuberculosis, in spite of the fact that the organism cultured in the laboratory was sensitive to all three antituberculous agents the patient had received. Another interesting point is that at no time until her collapse did her blood pressure fall below 110/80 and that on several occasions her electrolytes were normal. There is still no universal agreement on the treatment of spinal tuberculosis, and this led the Medical Research Council to set up a series of controlled trials; the first four reports have now been published (Medical Research Council 1973a, b, 1974a, b). These reports indicate that where facilities exist the 'Hong Kong' radical operation with chemotherapy gives the best result, but simple out-patient therapy with PAS and INAH may be relied on to give good results. Recent reports on the treatment of pulmonary tuberculosis with a combination of drugs including Rifampicin (East African/British Medical Research Councils 1973) for only six months gave very good results. Further study of this regime in the treatment of spinal tuberculosis is yet to be evaluated. REFERENCES East African/British Medical Research Councils (1973)Lanceti, 1331-1339 Jackson J W (1971) Postgraduate Medical Journal 47, 723-724 Medical Research Council (1973a) Journal of Bone and Joint Surgery 55B, 678 (1973b) Tubercle 54, 261 (1974a) Journal of Tropical Medicine and Hygiene 77, 72 (1974b) British Journal of Surgery 61, 853
Meeting 9 January 1974 The subject was Pain, and the speakers were Professor P D Wall (Cerebral Functions Group, Department of Anatomy and Embryology, University College London) and Dr Barry Wyke (Neurological Laboratory, Royal College of Surgeons, London). Meeting 8 May 1974 Dr D M L Doran (West Middlesex Hospital,
Isleworth) delivered his Presidential Address, entitled Is your Treatment really Necessary?
10 Meeting 28-29 June 1974 with the British Association for Rheumatology and Rehabilitation, the Swiss Societyfor Rheumatology, and the Heberden Society, at the University ofBristol
The following papers were read: Pain Threshold Analysis in Patients with Osteoarthrosis of the Hip Dr Susan L O'Driscoll (Department of Medicine, University ofBristol) and Dr Malcolm I V Jayson (Royal National Hospitalfor Rheumatic Diseases, Bath) REFERENCE O'Driscoll S & Jayson M I V (1974) British Medical Journal iii, 714-715
Lower Motor Neurone Weakness in Rheumatoid Cervical Myelopathy Dr J A Mathews (Department ofRheumatology, St Thomas' Hospital, London SEJ) REFERENCE Mathews J A (1975) Scandinavian Journal of Rheumatology 4, Suppl. 8, No. 064
Capillary Resistance and Back Pain Dr B J Sweetman and Dr J A D Anderson (Arthritis Research Unit, Guy's Hospital, London SEJ) REFERENCE Sweetman B J & Anderson J A D (1975) Rheumatology and Rehabilitation 14, 1
Meeting 12 February 1975 with the Society for Back Pain Research
The subject of the meeting was Criteria for the Diagnosis of Acute Intervertebral Disc Syndromes, and the principal speakers were Dr John A Mathews (St Thomas' Hospital, London SEI 7EH), Mr Martin Nelson (Leeds General Infirmary, Leeds 1) and Dr Marcia Wilkinson (Regional Neurological Unit, Eastern Hospital, London E9 6B Y). The meeting will be reported in Rheumatology & Rehabilitation, August 1975.
