Gut 1993; 34:567-569

Polymyositis associated with ulcerative colitis

S Chugh, J B Dilawari, I M S Sawhney, N Dang, B D Radotra, Y K Chawla

AbstractAn elderly woman with chronic ulcerativecolitis who developed proximal muscle weak-ness, increased serum creatine phosphokinaseactivity, and histological and electromyo-graphic abnormalities characteristic of poly-myositis is described. Treatment with corti-costeroids and 5-acetylsalicylic acid wasfollowed by a remission in bowel symptoms,improvement in muscle power, and reversal ofelectromyographic changes. An autoimmunelink between the two disorders seems likely.(Gut 1993; 34: 567-569)

Extraintestinal manifestations of inflammatorybowel disease are known to affect several organsystems.' Although 'granulomatous' myositis ismentioned occasionally as a rare complication ofinflammatory bowel disease,2 most reports onulcerative colitis do not mention any type ofmyositis as a complication or association of thedisorder.' 3 We have been able to find only fivecases of myositis"5 of different types complicat-ing ulcerative colitis, of which only two arepublished in English.4s Because of its rarity, wedocument the case of an elderly woman whodeveloped polymyositis as a complication of longstanding ulcerative colitis.

Departments ofMedicine, Hepatology,Neurology andPathology, PostgraduateInstitute of MedicalEducation and Research,Chandigarh, IndiaS ChughJ B DilawariI M S SawhneyN DangB D RadotraY K ChawlaCorrespondence to:Professor J B Dilawari,Department of Hepatology,Postgraduate Institute ofMedical Education andResearch, Chandigarh,160012, IndiaAccepted for publication31 August 1992

Case reportA 78 year old woman was admitted to hospital forinvestigation and management of diarrhoea andmuscle weakness. She had been having recurrentepisodes of diarrhoea associated with the passageof blood and mucus, low grade fever, and afeeling of exhaustion for the past 15 years. Eachepisode lasted for 7 to 10 days, followed by asymptom free period of 2-3 months. She hadnever visited hospital and was treated sympto-matically by general practitioners with anti-diarrhoeal drugs. During the past 15 days, shehad started having arthralgias that affectedmultiple joints and appreciable proximal muscleweakness of both lower and upper limbs. Thepatient was prescribed indomethacin (25 mgthrice daily) for arthralgia by her treating physi-cian. She had lost about 4 kg of weight over theprevious 2 weeks.

Physical examination at the time of hospitaladmission showed moderate pallor and milddehydration. The patient's pulse rate was96 bpm and the volume was slightly decreased.Her blood pressure was 100/60 mm Hg. Thesmall joints of the hands were tender to pressureand movement. Abdominal examination showedmild tenderness in the hypogastrium, and thepatient's spleen was palpable 2 cm below the leftcostal margin and was firm in consistency.Neurological examination showed weakness ofthe proximal muscles of the lower and upper

limbs and muscles of the trunk and flexors ofthe neck (grade 3/5). The superficial and deepreflexes were normal and her plantars wereflexor. Ocular fundi were normal.

Laboratory investigations showed a haemo-globin concentration of 78 g/l and a haematocritof 23%. The patient's total leukocyte count was8600/mm3 (8-6x 109/l), with neutrophils 85%,lymphocytes 13%, and eosinophils 2%. Theerythrocyte sedimentation rate was 67 mm in thefirst hour (Westergren) and reticulocyte count5%. A coagulogram was normal. Platelets were110000/mm3 (110x109/l). Serum Na was135 mmol/l and K 3-3 mmol/l. Blood ureawas 28 mg/dl (9 9 mmol/l) and serum creatinine1-0 mg/dl (88-4 mmol/l). Serum bilirubin was0-8 mg/dl (13-6 mmol/l); SGOT was 13 U/1,SGPT was 7 U/l and alkaline phosphatase was10 KAU. The total serum protein was 74 g/lwith an albumin concentration of 36 g/l, globulin38 g/l, and an albumin/globulin ratio of 0 95.The patient's urine was negative for protein andsugar, and microscopy showed 10-12 pus cells/high power field. Urine culture showed growthof Pseudomonas aeruginosa. Stools showed thepresence of red cells (+++++) but no ova orcysts, and culture grew Candida albicans. Uppergastrointestinal endoscopy showed features ofoesophagitis and erosive gastritis. A bariumenema showed extensive ulceration in the entirelarge bowel (Figs 1 and 2) and pseudopolypformation (Fig 3). Colonoscopy confirmed thepresence of multiple ulcerations and pseudo-polyps. Rectal biopsy specimen showed infiltra-tion of the lamina propria by inflammatory cellssevere mucodepletion, and cryptitis (Fig 4). Nogranuloma formation or vasculitis was noted. Abiopsy specimen from the left quadriceps muscleshowed focal intermyseal infiltration by lympho-cytes and occasional phagocytosis of degenera-

Figure 1: Barium enema showing extensive mucosalulceration (arrows) in the ascending, transverse. anddescending colon.

567

on Septem

ber 2, 2020 by guest. Protected by copyright.

http://gut.bmj.com

/G

ut: first published as 10.1136/gut.34.4.567 on 1 April 1993. D

ownloaded from

Chugh, Dilawari, Sawhney, Dang, Radotra, Chawla

.V ~ ~ .

Figure 4: Photomicrograph ofthe rectum showing intenseinflammatoty cell infiltration ofthe lamina propria,mucodepletion and cryptitis (arrow). (Original magnificationx240)

Figure 2: Barium enema showing ulceration (arrows) in thedescending and sigmoid colon.

tive muscle fibres (Fig 5). No evidence ofregenerative activity, vasculitis, or granulomawas seen. Electromyography of the right deltoidand right quadriceps muscles showed normalinsertional activity. No spontaneous activity wasobserved. Motor unit potentials were smallin amplitude and duration. The interferencepattern was full, and 30-40% polyphasia wasnoted (Fig 6). Myopathic motor unit potentialsand significant polyphasia were consistent withthe diagnosis of polymyositis.Nerve conduction studies were normal. The

patient's creatine phosphokinase activity wasincreased five fold (1010 U/1). Rheumatoid factorwas positive, and antinuclear antibodies werenegative.

COURSE AND MANAGEMENTThe patient was put on parenteral nutrition,oral prednisolone (40 mg once daily), and 5-acetylsalicylic acid (400 mg thrice daily). She alsoreceived 20 mg of prednisolone daily by enemaand 3 units of blood. In addition, she was givenciprofloxacin (500 mg twice daily) for 7 days forurinary tract infection and ketoconazole (200 mgtwice daily) for candidiasis. Her ulcerative colitis

Figure 3: Barium enemashowing pseudopolyps in thetransverse colon (arrows)and ulceration in theascending colon.

went into remission after 4 weeks and a sig-nificant improvement in her muscle power(grade 4/5) became noticeable after 12 weeks oftherapy. Repeat study of the electromyographicpattern 3 months later showed a distinct im-provement and this was accompanied by asignificant decrease in the serum creatininephosphokinase activity. The enlarged spleenregressed in size and became impalpable.

DiscussionSkeletal muscle involvement associated withinflammatory bowel disease was first reported in1970 by Spiro,9 who illustrated granuloma for-mation in striated muscle in a patient withCrohn's disease without giving any furtherdetails. In a subsequent study, Tydd and Dyer'0performed muscle biopsies in 15 patients withCrohn's disease in a search for granulomata.None of these patients, however, had anyclinical evidence of myopathy, and the musclehistology was normal in all of them. In 1976,Menard et al" reported granulomatous myositisand myopathy in a middle aged man withCrohn's disease. Two more cases were reportedsubsequently, one by Gilliam et al,'2 whodescribed an unusual vasculitic myositis in ayoung man with Crohn's disease, and another byHall et al,'3 who reported a non-specific localisedmyositis without histological evidence of musclegranulomata or vasculitis in a 32 year old womanwith long standing Crohn's disease.The earliest documentation of myositis com-

Figure 5: Photomicrograph ofthe left quadriceps muscleshowingfocal intermyseal lymphocytic infiltration andphagocytosis ofdegenerative musclefibres. (Originalmagnification xS50)

568

on Septem

ber 2, 2020 by guest. Protected by copyright.

http://gut.bmj.com

/G

ut: first published as 10.1136/gut.34.4.567 on 1 April 1993. D

ownloaded from

Polymyositis associated with ulcerative colitis 569

Figure 6: Electromyographof the right deltoid muscle ~showing myopathic motorunit potentials, polyphasia(above) and normal :; -interference pattern(below).

..

Compari'son of three reported cases of myositis i'n ulcerative colitis

Clini'cal and laboratory Kaneoka et al Bhiglee et atparameters (1990) (1987) Present case

Age at onset of myositis (y) 57 42 78Sex (MIF) F M FDuration of colitis prior to onset of 11 12 15

muscle weakness (y)Drugs before onset of myositis Steroids Steroids and No steroids or

sulphasalazine sulphasalazineSplenomegaly Massive, Absent Mild, regressed

regressed after after treatment oftreatment of myositismyositis

Creatine phosphokinase activity 301 U/I 262 U/l 1010 U/lAntinuclear factor + -

Rheumatoid factor + Not mentioned +

plicating ulcerative colitis came from Oshitaniet al,8 who described dermatomyositis andmononeuritis multiplex in ulcerative colitis andBasedow's disease. Four more cases, excludingours, have subsequently been reported to date,"7of which only two are reported in English."

Bhigjee et aA14recorded the case of a 44 year oldman who presented with ulcerative colitis of 12years' duration and muscle weakness of 3 years.He underwent colectomy for his bowel diseasebut despite surgery, the leg weakness progressedresulting in wasting of the quadriceps muscles.Kaneoka et a18 described polymyositis in a 63

year old womanpwho suffered from ulcerativecolitis and developed liver cirrhosis secondary topost-transfusional hepatitis. After a relativelysymptom free course of colitis for several years,she developed severe proximal muscle weaknessand generalised atrophy. She was observed tohave an enlarged spleen, which regressed afteradministration of steroids for polymyositis.

In both cases mentioned above and theone observed by us, myositis occurred after

long standing ulcerative colitis (Table). Serumcreatinine phosphokinase activity was raisedand there was no evidence of vasculitis orgranulomata on histology. In the absence of ahistory of sulphasalazine ingestion before theonset of myositis in two cases, one reported byKaneoka et al and the second observed by us,the role of this drug in the pathogenesis ofmuscledisease is doubtful. In addition, it is evident fromthe case described by Bhigjee et al' that presenceof active colitis is not essential for the progressionof the muscle disease. The disease entity seems toaffect both sexes and all ages since the conditionhas also been observed in a child.7 Regressionof splenomegaly after steroid therapy in twopatients (Table) and the presence of autoanti-bodies in the present patient as well as thatdescribed by Kaneoka et aV' suggest a possibleautoimmune link between ulcerative colitis andpolymyositis.

In the somewhat unrelated case of a 19 year oldman with ulcerative colitis and necrotisingcerebral vasculitis observed by Nelson et al,'4a biceps muscle biopsy specimen showed 'a fewnecrotic myofibres and mild perivascularinflammation'. Though not commented on bythe authors, the presence of myositis with orwithout vasculitis remains a distinct possibilityin their patient. Recognition of myositis as anextraintestinal manifestation of ulcerative colitisseems to be important for future documentationof this association.

1 Greenstein AJ, Janowitz HD, Sachar D. The extra intestinalcomplications of Crohn's disease and ulcerative colitis. Astudy of 700 patients. Medicine (Baltimore) 1976; 55: 401-12.

2 Glickman RM. Inflammatory bowel disease. In: Wilson JD,Braunwald E, Isselbacher KJ, Petersdorf RG, Martin JB,Fauci AS, Root RK, eds. Harrison's principles of internalmedicine. Vol 2, 12th ed. New York: McGraw Hill, 1991:1268-8 1.

3 Cello JP, Schneiderman DJ. Ulcerative colitis. In: SleisengerMH, Fordtran JS, eds. Gastrointestinal disease. Pathophysio-logy, diagnosis and management. 4th ed. Philadelphia: W BSaunders, 1989: 1435-76.

4 Bhigiee Al, Bill PLA, Cosnett JE. Ulcerative colitis andinterstitial myositis. Clin Neurol Neurosurg 1987; 89: 261-3.

5 Kaneoka H, lyadomi I, Hiida M, Yamamoto K, Kisu T,Tokunaga 0, Yamaguchi M. An overlapping case of ulcera-tive colitis and polymyositis. J Rheum 1990; 17: 274-6.

6 Evrard P, Lefebvre C, Brucher JM, Coche E. Mvositisassociated with ulcero-hemorrhagic rectocolitis. Apropos ofa case. Acta Gastroenterol Belg 1987; 50: 675-9.

7 Hernandez MA, Diez-Tejedor E, Barbado J, Morales C.Polymvositis and ulcerative colitis. A new form of associa-tion? Neurologia 1986; 1: 134-5.

8 Oshitani H, Wakabayashi Y, Sawaguchi Y, Koike H, YoshinoY. A case of dermatomyositis followed by multiple mono-neuritis, with the history of ulcerative colitis and Basedow'sdisease. ] Kyorin Med Soc 1981; 12: 47-53.

9 Spiro HM. Clinical Gastroenterology. Toronto: MacMillan,1970: 631.

10 Tydd TF, Dyer NH. Muscle biopsy in Crohn's disease. Lancet1974; ii: 1574-5.

11 Menard DB, Haddad H, Blain JG, Beaudry R, Devroede G,Masse S. Granulomatous mvositis and myopathy associatedwith Crohn's colitis. New Englj Med 1976; 295: 818-9.

12 Gilliam JH, Challa VR, Agudelo CA, Albertson DA, HuntleyCC. Vasculitis involving muscle associated with Crohn'scolitis. Gastroenterology 1981; 81: 787-90.

13 Hall MJ, Thomas WEG, Cooper BT. Gastrocnemius mvositisin a patient with inflammatory bowel disease. Digestion 1985;32: 296-300.

14 Nelson J, Barron MM, Riggs JE, Gutmann L, Schochet SS Jr.Cerebral vasculitis and ulcerative colitis. Neturology 1986; 36:719-21.

on Septem

ber 2, 2020 by guest. Protected by copyright.

http://gut.bmj.com

/G

ut: first published as 10.1136/gut.34.4.567 on 1 April 1993. D

ownloaded from