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NEUROLOGY NEUROSURGERY & PSYCHIATRY Editorial Journal ofNeurology, Neurosurgery, and Psychiatry 1991;54:285-287 285 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.54.4.285 on 1 April 1991. Downloaded from Joural of NEUROLOGY NEUROSURGERY & PSYCHIATRY Editorial The idiopathic inflammatory myopathies and their treatment The inflammatory myopathies are the largest group of As new knowledge has accumulated over the course of acquired myopathies of adult life and may also occur in the last 10 years, it has become increasingly clear that there infancy and childhood. They have in common the presence are distinct pathological and immunological differences of inflammatory infiltrates within skeletal muscle, usually between polymyositis on the one hand and dermato- in association with muscle fibre destruction. They can be myositis on the other, though in some cases there is clearly subdivided into those which are due to known viral, an overlap between the two conditions. In polymyositis bacterial, protozoal or other microbial agents and those in there is usually scattered necrosis of single muscle fibres which no such agent can be identified and in which which appear hyalinised in the early stages and are immunological mechanisms have been implicated.' The subsequently invaded by mononuclear phagocytic cells. latter group includes polymyositis, dermatomyositis and Regenerating fibres are usually seen singly or in small inclusion body myositis. The evidence for an autoimmune groups distributed focally and randomly throughout the aetiology consists of: 1) an association with other auto- muscle. The inflammatory cell infiltrate is predominantly immune diseases; 2) serological tests which reflect an intrafascicular (endomysial) surrounding muscle fibres altered immune state; and 3) the responsiveness of rather than in the interfascicular septa, though perivascular polymyositis and dermatomyositis, if not of the inclusion infiltrates may also be found; the cellular infiltrate consists body variety, to immunotherapy.2 Polymyositis may rarely mainly of lymphocytes, plasma cells and macrophages. occur in infancy (infantile myositis) but is commoner in Immunocytochemical studies using monoclonal antibody adult life, while dermatomyositis can occur both in chil- markers have shown that most of the lymphocytes and the dren and in adults and is often associated with other cellular infiltrates are activated T cells with a mixture of connective tissue diseases, with immune deficiency states CD8 + (suppressor/cytotoxic) and CD4 + (helper/ or with other systemic disorders; in middle or late life there inducer) cells, as well as macrophages.'3 14 Class 1 MHC is a statistical association with malignant disease. Both antigen expressed on the surface of non-necrotic muscle polymyositis and dermatomyositis can sometimes be drug- fibres which are being invaded by lymphocytes supports induced.3 the view that the muscle fibre damage is caused by http://jnnp.bmj.com/ Related disorders include sarcoid myopathy and cytotoxic T lymphocytes as these cells require the presence granulomatous myositis (which can only be distinguished of Class 1 MHC molecules on target cells to exert their from sarcoid myopathy by the absence of evidence of action.2 sarcoid in tissues and organs other than muscle), localised In dermatomyositis, particularly in childhood, isolated nodular myositis,4 eosinophilic polymyositis (which occurs muscle fibre necrosis is a rare finding; more often, portions as part of a systemic hypereosinophilic syndrome charac- of a muscle fasciculus may be necrotic and the lesions terised by eosinophilia, anaemia, hypergammaglobulin- resemble micro-infarcts. There is also a typical peri- aemia, cardiac and pulmonary involvement, skin changes, fascicular atrophy involving both type 1 and type 2 muscle on September 27, 2021 by guest. Protected copyright. peripheral neuropathy and encephalopathy5) and eosino- fibres. Microvascular changes, including capillary loss, philic perimyositis.67 Focal and relapsing forms of necrosis and thrombosis of capillaries, arterioles and eosinophilic myositis have been described,8 as has a venules accounting for the appearances resembling micro- form of angiopathic myositis, sometimes associated with infarction are also common.'5 16 Immunoglobulins and polyarteritis nodosa, Churg-Strauss vasculitis or complement components, including the C5b-9 membrane Wegener's granulomatosis.' Eosinophilic fasciitis may also attack complex, are commonly found in blood vessel walls, be confused with the myositic syndrome; in this condition suggesting that the condition is an immune complex vas- there is painful swelling and induration of the skin and soft culopathy.''8 Cellular infiltrates in this condition are tissues ofthe extremities and trunk leading to weakness and principally perimysial and perivascular and analysis of contractures in the limbs, often with a marked eosinophilia mononuclear cell subsets has shown a high percentage of B in the peripheral blood and hypergammaglobulinaemia.9 A cells and a high CD4 + /CD8 + T cell ratio in perivascular form of eosinophilic fasciitis presenting as a scleroderma- and perimysial sites. It is suggested that in this condition like syndrome, now called the tryptophan-eosinophilia- there is a helper T cell-dependent stimulation of B cells to myalgia syndrome, has also been reported in recent years in secrete immunoglobulins.'9 patients taking L-tryptophan for the treatment of depres- Inclusion body myositis, by contrast, is a distinct variety sion or insomnia'0 but is thought by some to be due to a of inflammatory myopathy, not generally associated with contaminant in the L-tryptophan rather than to a direct skin change, malignancy or collagen vascular disease, effect of the drug itself. A resemblance has been suggested though there have been occasional reports of an association between this condition and the so-called Spanish toxic oil with Sjogren's syndrome, scleroderma, immune syndrome." In some such cases a peripheral neuropathy thrombocytopenia, coeliac disease, sarcoidosis and malig- also occurs.'2 nancy.' The weakness is slowly progressive, usually pain- 286 Editorial ful, generally beginning in distal as well as proximal limb agents such as azathioprine, methotrexate and cyclo- J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.54.4.285 on 1 April 1991. Downloaded from muscles, occurring principally in males; usually the condi- phosphamide when given in addition to steroids. Cyclosp- tion is slowly progressive over a period of 10-20 years. orin A has proved successful in a few resistant cases, but Muscle biopsy reveals bluish granular inclusions dis- Grezard and others2' reported that a severe myopathy tributed round the edge of slit-like. vacuoles (rimmed resulted from cyclosporin treatment in a patient who had vacuoles) in muscle fibres; inflammatory cell infiltrates are previously undergone a renal transplant. Recently Engel predominantly endomysial and less frequently perimysial and Emslie-Smith' have found intravenous pulsed and perivascular in location. Electron microscopy in this methyl-prednisolone to be superior in its effects to oral condition invariably reveals intranuclear and cytoplasmic treatment in both polymyositis and dermatomyositis but masses of filamentous microtubules.20 While attempts at have suggested that such treatment should only be contin- viral isolation have been uniformly unsuccessful, Chou2" ued for a few weeks and should be followed by combined reported immunostaining for mumps virus antigens in the oral steroids given on an alternate-day basis with an nuclear and cytoplasmic inclusions in eight cases and appropriate immunosuppressive remedy for longer-term postulated that the condition is due to a chronic persistent maintenance treatment. Yet again, in some resistant cases mumps virus infection. However, Nishino and others22 total body irradiation or total lymphoid irradiation has have failed to confirm that finding. Hohlfeld and Engel23 been recommended and has been found successful by have recently reported results consistent with the hypo- Engel3" and others. More recently Cherin and others,32 thesis that the cytotoxic T cell response in this condition is writing from Paris, reported strikingly beneficial effects in directed against viral antigens, but the question of whether both polymyositis and dermatomyositis using intravenous it results from one or more specific viruses is still uncertain. immunoglobulin. Ten patients received lg per kg daily for For the pathological diagnosis of this condition, at least one two days each month while five received 0 5g per kg daily rimmed vacuole and at least one group of atrophic fibres for five days each month, the mean course of treatment must be seen per high-power field, and there must be an being four months. This treatment was given with pred- endomysialinflammatoryinfiltratewithlymphocytesinvad- nisone in 11 cases, methotrexate in five, and plasmapheresis ing non-necrotic muscle fibres as well as an electron- in one. Marked improvement was noted in 13 of the 15 microscopic demonstration of the typical filamentous cases so treated; the authors concluded that intravenous inclusions.20 immunoglobulin might well be used to replace or reduce Turning now to treatment, the response to steroid drugs steroid and immunosuppressive drug treatment in such and immunotherapy in inclusion body myositis has been cases. Finally, it should be mentioned that in der- almost invariably disappointing. While a few patients matomyositis, following
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