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Coeliac Disease, Vasculitis, and Cryoglobulinaemia

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Coeliac Disease, Vasculitis, and Cryoglobulinaemia Gut: first published as 10.1136/gut.13.2.112 on 1 February 1972. Downloaded from Gut, 1972, 13, 112-123 Coeliac disease, vasculitis, and cryoglobulinaemia WILLIAM F. DOE, D. EVANS, J. R. HOBBS, AND C. C. BOOTH From the Departments of Medicine, Morbid Anatomy, and Chemical Pathology, Royal Postgraduate Medical School, London SUMMARY Four patients are described with the association of adult coeliac disease, vasculitis, and cryoglobulinaemia. In each patient the cryoglobulinaemia was of the mixed type consisting of more than one immunoglobulin class. It is suggested that the mixed cryoglobulins represent circulating immune complexes and that their deposition in small vessels causes the vasculitis. This is supported by immunofluorescent studies of skin biopsies from one of these patients. The significance of these findings in the four adult coeliac patients is discussed. There is increasing evidence implicating immune SERUM C3 LEVELS mechanisms in the pathogenesis of coeliac disease Serum C3 levels were measured by a Mancini plate (Taylor, Truelove, Thomson, and Wright, 1961; technique using goat antihuman C3 (Hyland Rubin, Fauci, Sleisenger, and Jefferies, 1965; Ketz, Laboratories). The levels in 20 healthy control Kantor, and Herskovic, 1968; Hobbs and Hepner, subjects ranged from 100 to 200 mg/100 ml. 1968; Booth, 1970; Soltoft, 1970). In recent years we have seen four patients with adult coeliac disease, HAEMOLYTIC COMPLEMENT vasculitis, and a mixed Such an cryoglobulinaemia. Total haemolytic complement was measured by the http://gut.bmj.com/ association prompts further speculation on the method of Mayer, Croft, and Gray (1948). Crossed immunological mystery of coeliac disease. This immunoelectrophoresis (Laurell, 1965) was used for paper describes the clinical, biochemical, and the detection of degradation products of com- immunological features of the four patients with this plement (C3) in fresh serum from two patients hitherto unreported association. (cases 1 and 2). Materials and Methods ANTISERA TO HUMAN IgG, IgA, and IgM These antisera were prepared by injection of pooled on September 24, 2021 by guest. Protected copyright. CRYOGLOBULINS myeloma sera with Freund's complete adjuvant into These were isolated and purified by centrifugation rabbits. Antiserum to the C3 component of com- from serum separated at 370 and preserved for 12 plement was similarly prepared in rabbits from hours at 4°. The precipitate was then resuspended normal human sera. Antihuman fibrinogen (Hyland in cold buffered saline, washed four times at 40, and Laboratories) and fluorescein (FlTC) conjugated finally resuspended in phosphate-buffered saline. rabbit antihuman IgG, IgA, and IgM (Nordic To avoid losses from the small amounts of serum Laboratories), and rabbit antihuman gamma globu- available from two patients (cases 3 and 4) only lin (Burroughs Wellcome) were used for the one precipitation and resuspension cycle was per- immunofluorescent studies. formed. Immunoglobulin concentrations were measured by a modified Mancini plate technique SKIN BIOPSY (Mancini, Carbonara, and Heremans, 1965) on Skin biopsies were performed under local anaesthesia serum at 370 and then again on the supernatant at and frozen sections Sg, thick were prepared using a 40 after cryoprecipitation had occurred. In two cryostat. patients (cases 3 and 4) these estimations were carried out at 200 and at 4° respectively. To charac- GEL FILTRATION terize further the nature of the cryoglobulins, double Sephadex gel filtration was carried out using G200 diffusion in agar gel (Ouchterlony, 1958) and in a glass column 2-5 x 100 cm, bed volume 485 ml, immunoelectrophoresis were performed. with downward flow elution using 0-2M glycine/ Received for publication 2 November 1971. HCl buffer, pH 3.2, at a flow rate of 0.3 ml per minute. 112 Gut: first published as 10.1136/gut.13.2.112 on 1 February 1972. Downloaded from Coeliac disease, vasculitis, and cryoglobulinaemia 113 Three ml fractions were examined at 280 m,u using -;: an Hitachi Perkins-Elmer spectrophotometer. LATEX FIXATION This test was performed according to the method of Singer and Plotz (1956). SHEEP CELL AGGLUTINATION TEST This was performed according to the technique of Rose, Ragan, Pearce, and Lipman (1948). Patients Case reports of the four patients are detailed below and are summarized in Table I. CASE 1: ADULT COELIAC DISEASE, CRYOGLOBU- LINAEMIA, AND VASCULITIS V.A. attended Hammersmith Hospital in 1955 at the age of 42 with symptoms of anaemia (haemoglobin 6-6 g/100 ml), Raynaud's phenomenon, arthralgia of the knees, ankles, and fingers, and transient subcutaneous swellings on the forearms and hands. There was a blotchy, erythematous, papular rash which appeared periodically, lasting two to three days, and confined to the buttocks and lower limbs. The rash was precipitated by standing or prolonged exercise but was unaffected by cold. The rash subsided spontaneously and the anaemia responded http://gut.bmj.com/ to treatment with oral iron. He remained well until 1965, when both the skin rash and the anaemia recurred. Clinical examination now revealed a tall, pale man with an erythematous papular rash con- Fig. 1 Blotchy, papular rash seen on the thigh of fined to the buttocks and lower limbs (Fig. 1). case 1. on September 24, 2021 by guest. Protected copyright. Patient Clinical Data when Coeliac Disease Diagnosed Cryoglobulinaemia Course and Complications Features Jejunal Biopsy Faecal Fat Haemoglobin Serum Folate (g/24 hr) (g100 ml) (ng/ml) Case 1 Subtotal villous 13.5 8.0 1.0 Dermal vasculitis Rash and coeliac disease responded to M54(VA) atrophy Raynaud's, arthralgia, steroids; well on GFD' but rash returned lymphadenopathy when steroids ceased Case 2 Subtotal villous 28 10-8 2-0 ?Erythema nodosum, Sarcoidosis, positive thyroid and gastric F38(MC) atrophy retinal haemorrhage, antibodies; good response to GFD; retinal periphlebitis, deep well on maintenance steroids and GFD venous thromboses Case 3 Subtotal villous 25 13.1 1011 Erythema nodosum, Good initial response to GFD: F67(EB) atrophy dermal vasculitis deteriorated after gluten challenge; rash developed, unresponsive to steroids; Addison's disease; no autoantibodies; died with perforated gastric ulcers Case 4 Partial villous 14.3 7-0 1-5 Dermal vasculitis Good initial response to GFD; MS0(HS) atrophy3 later, following gluten challenge, coeliac disease and rash unresponsive to steroids; died with septicaemia Table Clinical summaries offour patients 'Patient taking regular folic acid supplements. 'GFD = gluten-free diet. 'Biopsy taken after 10 years' treatment with a gluten-free diet. Gut: first published as 10.1136/gut.13.2.112 on 1 February 1972. Downloaded from 114 William F. Doe, D. Evans, J. R. Hobbs, and C. C. Booth 1100 r balances averaged 13.5 g. Jejunal biopsy suggested the diagnosis of adult coeliac disease since on dis- secting microscopy the appearance was flat, and on histology there was subtotal villous atrophy. Treatment and progress Despite a satisfactory haematological response to 700 treatment with oral iron (ferrous sulphate 300 mg, CONC N three times daily) and folic acid (5 mg three times 6001 OF CRYOGLOBULIN daily), the rash became progressively more extensive _ MG /100Ml and by October 1967 involved the forearms as well 5001 as the buttocks and lower limbs. In addition, there 400 was a marked lymphadenopathy of the axillary, superficial inguinal, and femoral regions. A skin 300 biopsy now revealed vasculitis. There was peri- vascular oedema and a marked infiltration with 200 lymphocytes, plasma cells, and polymorphonuclear " 2 leucocytes. The lymph node biopsy, however, showed 100 the non-specific appearances of reactive hyperplasia. Cryoglobulins were now detected in the serum. 0 Li L-i L-i CASE 1 CASE 2 CASE 3 CASE 4 Immunological studies Fig. 2 Serum cryoglobulin concentrations. The cryoglobulin concentration was 260 mg/100 ml and consisted of mixed lgG (200 mg/100 ml) and There was pigmentation and purpura around both IgM (60 mg/100 ml) components (Fig. 2). This was ankles. confirmed using double diffusion in agar gel but no lines of precipitation formed against antisera to the Investigations C3 component of complement or to fibrinogen. http://gut.bmj.com/ Haemoglobin was 8.0 g/100 ml. Stained blood films When immunoelectrophoresis of the isolated cryo- showed both microcytosis and macrocytosis and protein was performed, the broad lgG arc suggested the bone marrow revealed mild megaloblastic that this cryoglobulin component was polyclonal changes with marked iron deficiency. Serum folate (Fig. 3). The electrophoretic strip of serum proteins was markedly reduced (1'0 ng/ml) but serum B12 showed an increase in the serum globulins (3.4 was normal (310 pg/ml). g/100 ml) but there was no evidence of a para- did Intestinal function tests showed a flat glucose protein and examination of concentrated urine on September 24, 2021 by guest. Protected copyright. tolerance test, the blood glucose rising only to 105 not reveal any Bence Jones protein. There was mg/100 ml from a fasting level of 80 mg/100 ml a marked increase in the ESR when measured at one and a half hours after 50 g of oral glucose. 370 (50 mm in the first hour) and compared to the D-Xylose excretion was 2.9 g in five hours after an ESR at 150 (24 mm in the first hour). The purified oral load of 25 g (normal 5 g/five hours). The daily cryoglobulin retained its cryoprecipitability after faecal fat excretion during two successive three-day being heated at 560 for 30 minutes. Fig. 3 Immunoelectro- phoresis of the cryoprecipitate from case 1 (in the welt) against specific anti IgG and IgM antisera. The broad IgG arc suggests that this cryoglobulin component is ---. ~polyclonal. Gut: first published as 10.1136/gut.13.2.112 on 1 February 1972. Downloaded from Coeliac disease, vasciulitis, and cryoglobulinaemia 115 035r The cryoprotein was then separated into its pure IgM and IgG components using a Sephadex G200 0.
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