reiter's syndrome in children. a case report and review

A SHORT NOTE

Reiter's Syndrome in Children

A Case Report and Review

George N. Lockie and Gene G. Hunder

A case of Reiter's syndrome in a 3-year-old boy is reported. No recurrence developed during the following 10 years. A review of previous cases of Reiter's syndrome in children suggests that although this disease is uncommon in children, the findings were similar to those seen in adults.

Reiter's syndrome is most commonly found in young men, is unusual in women and older men, and rare in children. The etiology is unknown, but previous sexual intercourse and bacillary dysentery have been considered precipitating causes (1). The diagnosis is based on the presence of the classic triad of arthritis, conjunctivitis and urethritis, although some patients who do not manifest the complete triad prob- ably have the same syndrome.

The first case of Reiter's syndrome in a youth was reported in 1918 (2) when a 16- year-old boy was described with arthritis, urethritis, conjunctivitis, and later iritis and keratitis. In 1947 the initial case of a preadolescent with Reiter's syndrome was published (3).

In the present report we described a

From the Mayo Graduate School of Medicine (University of Minnesota) , Rochester.

GEORGE N. LOCKIE, MD: Resident in Rheumatology, Mayo Clinic and Mayo Foundation. GENE G. HUNDER, MD: Division of Rheumatology and Internal Medi- cine.

Address reprint requests to: Section of Publica- tions, Mayo Clinic, Rochester, Minn 55901.

Submitted for publication Mar 8, 1971; accepted May 5. 1971.

3 Z/,,-year-old boy with Reiter's syndrome who has had no recurrences in the 10-year follow-up period. The clinical aspects of the previous cases reported in the literature are also summarized.

CASE REPORT A 3 2/12-year-old boy was seen at the Mayo Clinic

in December 1960, with conjunctivitis, arthritis, and dysuria. His eyes had become red 17 days previous- ly; there was no exudation or pain. Five days after onset, a moderately large subconjunctival hemor- rhage appeared in the left eye. Antibiotic drops, given for four days, did not help. Five days before admission, the patient developed spiking fevers up to 103 F. A transient erythematous rash was noted over the abdomen and right thigh. Three days later, he complained of marked pain in the shoul- ders, elbows, knees and ankles. His mother had to carry him to the bathroom because movement of the legs caused pain and he refused to walk. The left ankle and right knee appeared swollen. He was inactive in bed, tired easily, was anorectic and lost 4 pounds. On the day of entry, he also complained of prethral burning during urination and had several loose stools.

There was no family history of arthritis or conjunctivitis. No other family member had any recent illness.

The patient was 35 inches tall, and weighed 34 pounds, 14 ounces. The bulbar conjunctiva of both

Arthritis and Rheumatism, Vol. 14, No. 6 (November-December 1971) 767

LOCKIE & HUNDER

eyes I Y ~ S diffusely, deeply congested. The palpebral conjunctiva appeared normal. He had minimal generalized lymphadenopathy. The spleen tip was palpable below the left costal margin. The left ankle was slightly swollen and warm. The hemoglobin was 11.1 g/100 ml; leukocyte count, 5,200/ cu mm with 40% lymphocytes, 75% monocytes, 42% neutrophils, 8y0 eosinophils, and 2.5% baso- phils. Urinalysis showed grade 1 albumin and grade 2 leukocytes. The erythrocyte sedimentation rate was 73 mm in 1 hour (Westergren). The serum albumin was 2.58 g/ 100 ml serum, al-globulin 0.47 g, al-globulin 0.103 g; other serum protein fractions were normal. The AS0 titer, rheumatoid factor (Singer-Plotz), and LE clot test were normal or negative.

After aspiiin therapy began (blood level 19 mg/ 100 ml) , his symptoms improved. On reexamination two weeks later, the conjunctivitis was slightly less noticeable. Recurrent dysuria continued to be moderately severe. Joint pain and morning stiffness were less bothersome. Urinalysis was normal, and the erythrocyte sedimentation rate was 43.

Over the next two months, the joint pains, stiff- iiess and dysuria gradually subsided. The conjunctivitis continued for a total of three months, followed by two months during which he had inter- mittent episodes of eye redness lasting 2 to 3 days. Thereafter, he was seen occasionally for common childhood illnesses, but no subsequent symptoms related to his eyes, joints or genitourinary tract occurred. In May 1967, he developed muscular jerking and chorea. A tentative diagnosis of Sydenham’s chorea was made. A throat culture grew only normal flora; the antistreptolysin 0 titer was 250 (normal 0 to 50). Other tests that were negative or normal included hemoglobin, leukocyte count and differential, urinalysis, chest X-ray and electrocar- diogram. Penicillin was given and the patient improved over several months. No joint symptoms were present. He was examined in the fall of 1969 for an obsessive-compulsive neurosis which subse- quently improved.

REVIEW The literature was surveyed for reports

of Reiter’s syndrome in children. Arbitrari- ly, cases were selected for review only if the patients were found to have arthritis, conjunctivitis and urethritis beginning at age 16 or younger. Cases without specific de- scriptive information (4) or without the

complete triad (5, 6) were, therefore, not included.

The Table lists the major features of the 20 previous cases of Reiter’s syndrome in children, plus the present case. There were 17 boys and four girls. The cases were reported from North America and Europe.

Onset and Duration As noted in the Table, the age of onset

in the 21 patients was scattered throughout childhood. Two-thirds of the patients were nine years of age or older. Before the onset of the illness, the children were apparently in good health. Fourteen of the cases were sporadic, but in the other seven cases, more than one patient developed Reiter’s syndrome over a relatively short period either as members of a single family or as multiple cases in geographic proximity. Paronen’s (4) report of 344 cases of Reiter’s syndrome associated with a Shigella epidemic included four cases in children. A detailed description was given in only one, however. Zewi (3) reported three cases he observed to develop within a two-month period. In another instance, a father and son became sick at the same time (14) and in another report two brothers and their father developed Reiter’s syndrome (17).

Fifteen of the 21 cases reported in detail had diarrhea preceding other symptoms or early in the course of the disease. In one case bloody diarrhea was present (4). Ma- laise, anorexia, nausea, fever (9, 12, 13) and weight loss (8) also were described. A transient skin rash on the trunk was noted in one case (9), as well as in our patient.

Although conjunctivitis was the most common initial symptom, there was no consistent order in which the findings developed. Arthritis usually developed after one or more other symptoms. In 10 of the 21, the initial symptom was conjunctivitis (3, 4, 6, 9, 15, 17), in four, urethritis (2, 8, 12, 13) and in three, arthritis (9, 14, 16);

768 Arthritis and Rheumatism, Vol. 14, No. 6 (November-December 1971)

REITER’S SYNDROME

Rdsumd of Cases of Reiter’s Syndrome in Children

Age of Dura:ion of Author and onset Init ial organ symptoms

Case reference number (yr) Sex system involved Diarrhea (mo)

1 Junghanns (2) 2 Koster and Jansen (6) 3 Zewi (3) 4 Zewi (3) 5 Zewi (3) 6 Florman and Goldstein (7) 7 Paronen (4) 8 Corner (8) 9 Henckel (9)

10 Henckel (9) 11 Henckel (9) 12 Cantarutti (10)

,13 Neirnann e ta / (11) 14 Jacobs (12) 15 Margileth (13) 16 Gough (14) 17 Moss (15) 18 Herman (16) 19 Davies eta / (17) 20 Davies eta / (17) 21 Lockie and Hunder

16 15 4

16 14 4 3 9 9 3 3

10 2 9 6

15 10 13 11 17 3

M M M F M M M M F F F M M M M M M M M M M

Genitourinary 0 Eye + Eye + Ey a + Eye + Genitourinary; eye + Eye + Genitourinary 0 Eye + Gen i tour i na ry ; eye 0 Joint 0 Genitourinary; eye + Genitourinary ; eye + Genitourinary + Ge n i tou r i nary + Joint 0 Eye + Joint 0 Eye + Eye + Eye +

2% 1-2

2 3 4 1% 4 8 2 2 2 3 3 1% 5 3 1

10 1 1 3

four others developed urethritis and conjunctivitis simultaneously (7, 9-1 1). All three major symptoms usually developed within a 1- to 2-week period, but occasionally all were manifested only after several weeks (9, 14, 17).

The duration of the initial attack in the cases with adequate description ranged from 1 (15, 17) to 10 months (16), with aa average of slightly over three months.

0 p ht ha I m ologic Symptoms Conjunctivitis was bilateral in all cases.

Although at times it was severe, with bleph- arospasm and photophobia (7), i t was frequently mild. It was often variable in degree or was recurrent during the illness or occasionally later (3, 7, 12, 15, 17). Iritis (3, 10, 17) and keratitis (3, 7, 10, 17) were also reported commonly and scarring of the corneas occurred (1, 7).

Genitourinary Symptoms Although in adults with Reiter’s disease

the urethral discharge is often readily ap- parent, in children it was usually described as scant. Six children had frank urethral discharge (3, 4, 11, 15-17); others had pain on urination or urinary frequency. In one instance, pyuria lasted three months (8). There were no late genitourinary compli- cations.

Articular Manifestations The degree of arthritis varied from mild

to severe and was generally the most pro- longed symptom. In 11 patients, the arthritis was present less than two months, but four of the patients had arthritis lasting five months or longer (3, 8, 16, 17). In three patients, only one joint was involved (9, 12, 17). Joints of the lower extremities were involved in all 21 cases. The knee was in-


LOCKlE & HUNDER

volved in 17, the ankle in 12, metatarso- phalangeal joints in six, elbows in five, metacarpophalangeal joints in four, hip in four, shoulder in three and wrist in two. The joint involvement was frequently asym- metric (4, 6), and the joints were commonly described as being tender, red, warm and swollen. Symmetrical joint involvement, however, occurred in eight (1, 4, 8, 17). At times, the severity of symptoms in a joint fluctuated (8, IS), and joint symptoms were described as migratory in one case (16). Heel pain occurred (9 and X-rays showed changes of periarticular demineralization (13, 14), periostitis (14) and a bony erosion (14). A synovial fluid specimen was described as purulent, with a leukocyte count of 17,OOO/cu mm, all polymorphonuclear cells (7).

Mucocutaneous Lesions Two patients had keratodermia blenor-

rhagia (10, 14) and three had circinate balanitis (7, 10, 12). All lesions apparently healed without special treatment.

Laboratory Findings Anemia, slight leukocytosis and pyuria

were common. Shigella agglutinins in the blood were present in 8 of 12 cases tested (4, 6, 7, 10, 12); one patient who had no diarrhea also had Shigella agglutinins (10). Shigella flexneri were cultured from the stool of one patient (17) and Salmonella enteritidis organisms were cultured from the stool of another (11).

Treatment Several forms of treatment were given.

Penicillin (7), tetracycline, ampicillin and sulfa drugs (7, 13) were used with no ap- parent improvement. Adrenocorticoid therapy, given systemically (9-11, 14, 17) or in topical eye solutions (12, IS), appeared to help in some cases. Aspirin did not help

in some cases but was felt to be beneficial in others (13, 14, 16).

Recurrences Recurrences were infrequent; however,

only five patients were followed longer than one year. In one patient, a relapse of two months’ duration occurred one year after the initial attack and included symptoms of arthritris, urethritis, and keratodermia blenorrhagica (14). Another patient had a brief relapse with conjunctivitis, arthritis and urethritis after 9 months, but none over the next five years (16). A third had fleeting arthralgias in various joints in the months subsequent to the initial attack and mild swelling of the right ankle at four months (17). Including the present case, three patients were followed for 5 to 10 years without relapse (13, 16).

DISCUSSION Although the number of cases of Reiter’s

syndrome reported in children is relatively small, the illness appears to have most of the features seen in adult cases. In addition, the simultaneous occurrence of cases in par- ents and children (4, 6, 14) suggests that similar etiologic factors (presently unknown) are important. No specific reason could be found to explain completely the rarity of Reiter’s syndrome in children. Lack of sexual activity may account, in part, for the low incidence, but even in Paronen’s (4) 344 cases of “postdysenteric Reiter’s syndrome,” only about 1% OC-

curred in children. All major manifestations described in

adult cases have been observed in children, but not some rare manifestations such as carditis and valvulitis (4, 18), pleuritis (4). neuritis (4) and nephritis (4).

The incidence of diarrhea in the children (15 of 21) was higher than the 10 to 30% usually recorded in adult series (1).

Ocular involvement tended to be promi-


REITER’S SYNDROME

nent in the children. Iritis or keratitis or both occurred in 19% (4 of 21) (3,7, 10, 17). This incidence is somewhat higher than the 10% described in the adult series (19). On the other hand, mucocutaneous lesions which have been reported in up to 80% of adults (19) were seen in only 5 of 21 cases. The importance of the above differ- ences between adults and children is unknown and could merely reflect the small number of pediatric cases reported.

As in adults, arthritis generally tended to dominate the clinical picture. The large joints of the lower extremities were most commonly affected. Although radiologic changes were noted, no residual joint dam- age or spondylitis was reported, in contra- distinction to many adult cases (20-22). In one patient, asymptomatic slight thickening of the knee joints was noted at the end of one year (8). Again, the lack of observed permanent joint changes may relate to the small number of cases followed over long periods.

In general, the patients recovered quick- ly from the illness, but follow-up data in children was sparse. As noted, only 3 of 21 children had recurrences (14, 16, 17), but only five were followed for more than one year (13, 14, 16, 17). This is different from the results of long-term follow-up studies of adults in which the majority have recurrences or persistent symptoms (21, 23). Although it is interesting to speculate about a possible relationship between the chorea our patient had and his Reiter’s syndrome, there is no evidence that they were related.

In the reported cases, symptomatic therapy was usually satisfactory. Treatment guidelines for adults (1, 24) would appear to apply to children as well. Because of the association, in some instances, of Reiter’s syndrome with Shigella infections, stool cul- tures for this organism should be obtained

and appropriate treatment given if neces- sary. Studies for mycoplasma and Bedsonia (Chlamydia) organisms were not made in the reported cases but could be considered in future cases in the light of recent inves- tigations (24, 25). Other forms of arthritis which may cause similar symptoms should also be excluded (26).

REFERENCES

1. Ford DK: Reiter’s syndrome, Arthritis and Allied Conditions: A Textbook of Rheu- matology. Seventh edition. Edited by JL Hollander. Philadelphia, Lea & Febiger,

2. Junghanns 0: Ein weiterer Fall von Ure- thritis non gonorrhoica und septischer Allgemeinerkrankung: Beitrag zur Spiro- chaetosis arthritica Reiter. Deutsch Med Wschr 44:1304-1305, 1918

3. Zewi M: Morbus Reiteri. Acta Ophthal (Kobenhavn) 25:47-60, 1947

4. Paronen I: Reiter’s disease: a study of 344 cases observed in Finland. Acta Med Scand

5. Wright V: Arthritis associated with venereal disease: a comparative study of gonococcal arthritis and Reiter’s syndrome. Ann Rheum Dis 22:77-89, 1963

6. Koster MS, Jansen MT: Morbus Reiteri. Nederl T Geneesk 90:483485, 1946

7. Florman AL, Goldstein HM: Arthritis, conjunctivitis, and urethritis (so-called Reiter’s syndrome) in a four-year-old boy. J Pediat 33:172-177, 1948

8. Corner BD: Reiter’s syndrome in childhood. Arch Dis Child 25:398-403, 1950

9. Henckel H: Das Reitersche Syndrom bei Kindern und seine Therapie. Z Kinder- heilk 74:303-319, 1954

10. Cantarutti F: La sindrome di Reiter nell infanzia. Ada Paediat Lat ?:273-285, 1954

11. Neimann N, Pierson M, Ginsbourger N: Syndrome de Fiessinger-Leroy-Reiter d6- termink par le bacille de gaertner chez le nourrisson. Rev Med Nancy 84302-305, 1959

1966, pp 1001-1015

(Sup@) 212~1-112, 1948


LOCKlE & HUNDER

12. Jacobs AG: A case of Reiter’s syndrome in childhood. Brit Med J 2:155, 1961

13. Margileth AM: Reiter’s syndrome in children: case report and review of literature. Clin Pediat (Philadelphia) 1:148-151, 1962

14. Gough KR: Reiter’s syndrome in father and son. Ann Rheum Dis 21:292-294, 1962

15. Moss IS: Reiter’s disease in childhood. Brit J Vener Dis 40:166-169, 1964

16. Herman M: Reiter’s syndrome in a 13-year- old boy. Wisconsin Med J 65:195-196, 1966

17. Davies NE, Haverty JR, Boatwright M: Reiter’s disease associated with shigellosis. Southern Med J 62:lOll-1014, 1969

18. Rodnan GP, Benedek TG, Shaver JA, et al: Reiter’s syndrome and aortic insufficiency. JAMA 189:889-894, 1964

19. Sharp JT: Reiter’s syndrome: a review of clinical features and studies on etiology. Med Clin N Amer 45:1325-1336, 1961

20. Weinberger HW. Ropes MW, Kulka JP, et al: Reiter’s syndrome, clinical and patho- logic observations: a long term study of 16 cases. Medicine (Balt) 41:35-91, 1962

21. Sairanen E, Paronen I, MZhonen H: Reit- er’s syndrome: a follow-up study. Acta Med Scand 185:57-63, 1969

22. Good AE: Reiter’s disease and ankylosing spondylitis. Acta Rheum Scand 11:305-317, 1965

23. Csonka GW: Recurrent attacks in Reiter’s disease. Arthritis Rheum 3:164-169, 1960

24. Ford DK: Reiter’s syndrome. Bull Rheum Dis 20:588-591, 1970

25. Schachter J, Barnes MG, Jones JP Jr, et al: Isolation of Bedsoniae from the joints of patients with Reiter’s syndrome. Proc SOC Exp Biol Med 122283-285, 1966

26. Fink CW: Gonococcal arthritis in children. JAMA 194~237-238, 1965


reiter's syndrome in children. a case report and review

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