A patient in whom primary Sjogren's syndrome and inappropriate antiduretic hormone secretion were associated is reported. This is the first report of such an association. The possible pathophysiological mechanisms are discussed and vasculitis proposed as the underlying pathogenetic mechanism

de Villiers, W.J.S.

Klemp, P

English

AJOL - African Journals Online

12. Jessop S, Myers OL. Systemic lupus erythematosus in Cape Town. S AfrMedJ 1973; 47: 222-225.13. Siegel M, Lee SL. The epidemiology of systemic lupus erythematosus.Semin Arrhn'/is Rheum 1973; 3: 1-9.14. Fessel1- Systemic lupus erythematosus in the communiry. Arch Intem Med1974; 143: 1027-1035.15. Siegel M, Holley HL, Lee SL. Epidemiologic studies on systemic lupuserythematosus: comparative data for New York City and Jefferson County,Alabama, 1956-1965. Ar/hri/isRheum 1970; 13:802-811.16. Otieno LS, Wairagu SG, Waweru HW. Systemic lupus erythematosus atSAMJ VOL. 79 19 JAN 1991 103Kenyana National Hospital, 1972-1984. East Afr MedJ 1985; 62: 391-396.17. Bahemuka M, Bhau KM. Transient retinopathy in uncomplicated case ofsystelDlc lupus erythematosus. East Afr MedJ 1980; 57: 500-504.18. I?ernen fP'. ~onnanf CD, MODDier A, Ducloux M. Lupus erythemateux31gu disselDlDe chez I Mncain de race noire. Bull Soc Med Afr Noire Long Fr1976; 21: 65-70.19". Ransome OJ, Thomson PD. Systemic lupus erythematosus with nephritis inchildren: a repon of 6 cases. S Afr MedJ 1986; 69: 629-633.20. Walpon MJ, Black CM, Batchelor JR. The immunogenetics of SLE. ClinRheum Dis 1982; 8: 3-21.Primary Sjogren's syndrome associated withinappropriate antidiuretic hormone secretionA case reportW. J. S. DE VILLlERS, P. KLEMPSummary Case reportA patient in whom primary Sjogren's syndrome and inappro-priate antiduretic hormone secretion were associated isreported. This is the first report of such an association. Thepossible pathophysiological mechanisms are discussed andvasculitis proposed as the L:nderlying pathogeneticmechanism.SAtf Med J 1991; 79: 103-104.Sjogren's syndrome is an auto-immune disorder with a pre-dilection for multisystem involvement.! Xerophthalmia andxerostomia from a destructive mononuclear infiltration of thelacrimal and salivary glands are characteristic. Central nervoussystem (CNS) involvement includes recurrent aseptic meningo-encephalitis, necrotisin~ spinal arteritis and unifocal and multi-focal cerebral disease. ,3 Inappropriate secretion of argininevasopressin (SIADH) is a form of hyponatraemia in whichthere is an elevated level of antidiuretic hormone (ADH) in-appropriate to any physiological stimuli present that normallyaffect ADH secretion.4 Among the causes are malignant'tumours, chest infections and CNS disturbances.4A patient in whom primary Sjogren's syndrome and inappro-priate ADH secretion were associated is reported. This is thefirst report of such an association. The possible pathophysio-logical mechanisms are discussed.Rheumatology Unit, Department of Internal Medicine,University of Stellenbosch and Tygerberg Hospital, Parow-valiei, CP .W. J. S. DE VILLIERS, M.B. CH.B.P. KLEMP, M.D., F.C.P. (S.A.) (Present address: Queen ElizabethHospital, Rotorua, New Zealand)Acxxpted 30 March 1990.A 56-year-old woman presented to hospital with a generalisedconvulsion. She was not hypovolaemic and, apart from adecreased level of consciousness, the neurological system wasnormal.The serum sodium level was III mmol/I (normal 133 - 146mmol/l), chloride 74 mmol/I (normal 96 - 106 mmol/l), urea2,1 mmol/l (normal 3,3 - 6,5 mmol/l), uric acid 0,18 mmol/I(normal 0,20 - 0,45 mmol/l) and osmolaliry 237 mmol/kg H20(normal 275 - 290 mmol/kg H 20). Serum potassium, glucoseand creatinine values were normal. Urinalysis revealed a normalsediment, no proteinuria or glycosuria and a sodium content of63 mmol/I, potassium 123 mmol/l, chloride 94 mmol/l andosmolaliry 488 mmol/kg H20 (normal 50 - 1400 mmol/kgH20). The full blood count was normal, the erythrocytesedimentation rate was 100 mm/1st h (Westergren) and theC-reactive protein 49 mgll (normal < 10 mgll).Blood culture and toxicology screening for phenothiazines,ethanol, paracetamol, barbiturates and benzodiazepines werenegative. An ECG, chest radiography, blood gas analysis,thyroid function tests and the cortisol level were norm~ andscreening for porphyrin in urine and stool was negative.Computed tomography of the brain and cerebrospinal fluidexamination were non-contributory, while electro-encephalo-graphy showed nonspecific diffuse changes compatible with ametabolic encephalopathy.SIADH was diagnosed and fluid intake restricted to 500ml/24 h. In addition, 200 ml of 5% saline was administeredtwice 8-hourly. The serum sodium level rose to 126 mmol/lwithin 24 hours and the patient became fully conscious.Serum and urine electrolyte values and osmolaliry returned tonormal and remained so.Further enquiry revealed a 3-year history of dry eyes andmouth and swelling of the left parotid gland. There was nohistory of skin rashes, Raynaud's phenomenon, oral or genitalulcers, photosensitiviry, serositis or musculoskeletal symptoms.The patient denied any drug intake. No salivary gland enlarge-104 SAMJ VOL 79 19JAN 1991ment or lymphadenopathy were present but an absence ofpooling of saliva in the floor of the mouth was noted. Slit-lamp examination of the eyes showed a diminished tear ftlmand punctate areas of epithelial loss were demonstrated with1% rose bengal drops. Liver function tests, serum creatinekinase, aldolase, calcium, magnesium, phosphate and comple-ment levels was normal. Cryog~obulins were negative and ahyperglobulinaemia of 42 g/l with increased IgA and IgGlevels were demonstrated. .The rheumatoid factor was pOsitive on three and the Rose-Waaler test on two occasions (titres of 1:80 and 1:20). Anti-nuclear antibodies ranged from 1:10 to 1:1280 (speckledpattern) and anti-Sm and anti-RNP antibodies were positiveon three occasions. Anti-double-stranded DNA, anti-Ro (SAA)and anti-La (SSB) antibodies were persistently negative. Alabial salivary gland biopsy revealed focal mononuclear cellinltltration. The patient was treated with artificial tears andhas remained well for 26 months.DiscussionSIADH was first described by Schwartz er a1. 5 Diagnosticcriteria for this syndrome have subsequently evolved andinclude: (I) hypotonic hyponatraemia; (i/) urine osmolalityhigher than would be anticipated for the degree of hyponatrae-mia; (iil) normal renal, adrenal and pituitary function; (iv)excretion of appreciable quantities of sodium in the euvolaemicpatient; (v) absence of hypovolaemia or dehydration; (VI)absence of a condition associated with generalised oedema orascites; and (viI) correction of both the hypotonic hyponatrae-mia and natriuresis by severe fluid restriction.4 Our patientfulItlled all these criteria and was on no medication, such asdiuretics and non-steroidal anti-inflammatory agents, whichcan cause a SIADH-like picrure.6 The hypo-uraemia andhypo-uricaemia present in our patient have been well describedin SIADHYIn a sm~ number of patients with SIADH no cause can beidentified, but Martinez-Maldonad09 advises caution in diag-nosing 'idiopathic' SIADH, since the syndrome might precedeovert disease, specifically cancer, by months or years.The diagnosis of primary Sjogren's syndrome (sicca syn-drome) was made by the triad of keratoconjunctivitis sicca,xerostomia and mononuclear cell inltltration in a labial salivarygland biopsy, and the clinical and serological exclusion ofother connective tissue disease. lO•lI When diminished tearsecretion and punctate keratitis coexist, keratoconjunctivitissicca is considered present. No entirely satisfactory clinical orlaboratory tests are as yet available for the diagnosis of the oralcomponent of Sjogren's syndrome. The most reliable signs areprobably the absence of pooling of saliva in the floor of themouth and unilateral, episodic parotid gland enlargement,both of which were present in our patient. lO Focal lymphocyticinltltrates in minor salivary glands are' seen in over 70% ofpatients with Sjogren's syndrome. lOA computer-assisted search of'medical publications revealedno previous report of an association between primary SjOgren'ssyndrome and SIADH. This association could be entirelyfortuitous. The rarity of idiopathic SIADH and the patient'swell-being 26 months after diagnosis suggest that there is atrue association between the two conditions.Possible pathogenetic mechanisms to explain CNS mani-festations of Sjogren's syndrome include vasculitis, directmononuclear cell inltltration of CNS tissue, antineuronal auto-antibodies and cerebral vasospasm.2 An association betweenperipheral vasculitis, CNS involvement and anti-Ro (SSA)antibodies has been described in primary Sjogren's syndromeYVasculitis probably accounts for a significant proportion ofCNS complications in Sjogren's syndrome. lO The diversity ofCNS manifestations in Sjogren's syndrome is comparable tothat of systemic lupus erythematosus and it is conceivable thatthe underlying pathogenetic; mechanisms, including vasculitis,may be similar..12 Systemic lupus erythematosus vasculitis haspreviously been reported as a cause of SIADH, with immune-mediated inflammation postulated to have caused release of.ADH from the neighbouring neurohypophysis.9In similar fashion, we propose a direct association betweenprimary Sjogren's syndrome and the subsequent developmentof SIADH in our patient, with vasculitis as the possibleunderlying pathogenetic mechanism.REFERENCES1. Shearn M. Sj6gren's SyndrlJ11le. Philadelphia: WE Saunders, 1911: 15.2. Alexander EL, Provost IT, Stevens ME, Alexander GE. Neurologic compli-cations of primaty Sjogren's syndrome. Medicine (Balcimore) 1982; 61:247-257.3. Sigal LH. The neurologic presentation of vasculitic and rhewDatologicsyndromes. Medicine (Baltimore) 1987; 66: 157-180.4. Goldherg M. Hyponatremia. Med Clin Norch Am 1981; 65: 251-269.5. Schwanz WE, Bennen W, Curelop S, Barner FC. A syndrome of renalsodium loss and hyponatremia probably resulting from inappropriate secre-tion of antidiuretic hormone. AmJ Med 1957; 23: 529-542.6. Bidani A. Electrolyte and acid-base disorders. Med Cli,. Norch Am 1986; 70:1013-1035.7. Decaux G, Genene F, Mockel J. Hypouremia in the syndrome ofinappro-priate secretion of antidiuretic hormone. Ann I,.eem Med 1980; 71: 716-717.8. Beck LH. Hypouricemia in the syndrome of inappropriate secretion ofantidiuretic hormone. N EnglJ Med 1979; 301: 528-530.9. Martinez-Maldonado M. Inappropriate antidiuretic hormone secretion ofunknown origin. Kidney Ine 1980; 17: 554-567.10. Fox RI, Howell FW, Bone RC, Mitchelson P. Primaty Sjogren's syndrome:clinical and immunopathological features. Semi,. Archricis Rheum 1984; 14:77-IOS.I I. Fox RI, Robinson CA, Curd JG, Kozin F, HoweJJ FV. Sjogren's syndrome:proposed criteria fQr classification. Archn'cis Rheum 1986; 29: 577-585.12. Ellis SG, Verity MA. Central nervous system involvement in ~ystemic lupuserythematosus: a review of neuropathologic findings in 57 cases, 1955-1977.Semin Archricis Rheum 1979; 8: 212-221.

Primary Sjogren's syndrome associated with inappropriate antidiuretic hormone secretion A case report

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Primary Sjogren's syndrome associated with inappropriate antidiuretic hormone secretion A case report

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