Pulmonary fibrosis and other clinical manifestations of small vessel vasculitis in a family with seropositive juvenile rheumatoid arthritis

2001 ◽  
Vol 33 (1) ◽  
pp. 65-70 ◽  
Author(s):  
Julia Rohayem ◽  
W. Leupold ◽  
K.-D. Paul ◽  
M. Gahr

Summary. Reactive arthritis includes inflammatory non-purulent diseases of the joints that develop within 2-4 weeks after intestinal or urogenital infection due to immune disorders. Given the nonspecificity of the clinical manifestations of reactive arthritis, its similarity to other rheumatic diseases, which have a more unfavorable course and prognosis, it must be recognized that the problem of reactive arthritis in children remains relevant and on many issues not developed. Objective: this is to identify the characteristics of the debut and currents of reactive arthritis in accordance to etiology in children today. Materials and methods: There were observed 81 children with a reactive arthritis in the age of from 2 till 17 years have been examined. During specification the nosology of articular pathology were used clinical, laboratory and instrumental investigations, identified specific antibodies to Chlamydia, Mycoplasma, Iersiniya, Ureaplazma, Citomegalovirus, Herpes simplex virus, Epstein-Bar’s virus by ELISA. Results: For reactive arthritis chlamydial etiology characterized by loss of large and medium-sized joints of the lower limbs, which often is accompanied by a brief morning stiffness and rapid emergence of transient regional hypotrophy of muscles. Feature ureaplazmial reactive arthritis is the formation of bursitis in the heel and tendinitis. Reactive arthritis, which is accompanied by elevated titers to ASL-O, had different polymorphisms of articular manifestations of the syndrome and to a certain degree of similarity with juvenile rheumatoid arthritis. Reactive arthritis of not specified etiology has a number of the general features with others reactive arthritis and it is characterized by rather good-quality current, long conservation of function of joints and low laboratory activity. Conclusions: The clinical manifestation of the disease and the nature of its course to some extent depend on the etiological pathogen of arthritis. The most unfavorable variants of reactive arthritis are arthritides caused by Chlamydia and Mycoplasma. Regardless of the pathogen, the infectious factor can be assessed as a trigger for the development of juvenile rheumatoid arthritis. The presence of recurrent reactive arthritis is the basis for re-differential diagnosis and reclassification of reactive arthritis in favor of juvenile rheumatoid arthritis, despite the low degree of inflammatory activity of the disease and ANA and HLAB27 negativity.


PEDIATRICS ◽  
1993 ◽  
Vol 92 (2) ◽  
pp. 295-296
Author(s):  

Chronic, nongranulomatous iridocyclitis is an important complication of juvenile rheumatoid arthritis (JRA).1-3 First reported by Ohm in 1910, the association between iridocycitis and JRA has become well established. The intraocular inflammation referred to as iridocyclitis primarily affects the iris and ciliary body. Overall, the reported incidence of iridocyditis varies from 2% to 21% in children with JRA.4 The morbidity of iridocydlitis includes cataracts, glaucoma, band keratopathy, and loss of vision.5,6 Diagnosis of early iridocycitis is usually not possible by routine direct ophthalmoscopy. Sitlamp examination detects the signs of active anterior chamber inflammation. Guidelines for the schedule of routine serial sitlamp examination are suggested for early detection of iridocycitis. The presentation of eye involvement in JRA may be asymptomatic or of an insidious onset. The outcome has improved in the past 20 years. The majority of children have a relatively good visual prognosis if the iridocycitis is detected and treated early.6 RISK FACTORS FOR IRIDOCYCLITIS Articular Features The diagnosis of JRA describes a heterogeneous group of arthritic conditions with onset of disease before age 16 years. There are three major subtypes of JRA: systemic onset, polyarticular onset, and pauciarticular onset, defined by the clinical manifestations in the first 6 weeks of the disease.7 Fewer than 2% of children with systemic-onset JRA have iridocycitis.4,5 Children with polyarticular disease are at moderate risk, with 7% to 37% incidence of iridocyditis. The majority of children with iridocycitis have pauciarticular disease.1-3,5 The onset of the iridocyclitis may precede the onset of the arthritis in approximately 6% of cases.


Author(s):  
Rajkumar Kannan ◽  
Muthusubramanian Chandrasekar ◽  
Sridhar Venu ◽  
Jayakalyani Vijayananth

<p class="abstract"><strong>Background:</strong> Cutaneous small-vessel vasculitis (CSVV) is a group of disorder which is characterised by involvement of capillaries, arterioles and venules. CSVV can be idiopathic or primary, or secondary to infection, drugs or as a part and parcel of underlying systemic disease. The aim of our study is to find out the etiological factors, treatment options and their outcome in CSVV.</p><p class="abstract"><strong>Methods:</strong> We analysed 75 cases of CSVV out of patients who attended Dermatology OPD, in a tertiary care-centre from April 2017 to March 2018.The study design was descriptive study. A detailed history taking, thorough clinical examination and appropriate relevant investigations including biopsy were done for all the patients fulfilling the inclusion criteria and exclusion criteria.<strong></strong></p><p class="abstract"><strong>Results:</strong> A sample size of 75 patients (53 women and 22 men) were included in the study. Their mean age was found to be 25 years (range 18-40). The following etiological factors were made out in our study: Benign isolated (40) patients, (53%), infective etiology (14) patients, (19%), vasculitis in background of ANA/dsDNA/ANCA positivity (12) patients, (16%), drug induced (9), patients (12%). The main clinical manifestations of CSVV in our study were found to be the following viz, palpable purpura in all 75 patients (100%), fever &amp; malaise in 30 patients, (40%), ulcers in 30 patients (40%) arthritis/arthralgia in 15 patients, (20%). After a median follow up of 6 months, complete recovery was observed in all patients, although relapses occurred in 8 patients (11%).</p><p><strong>Conclusions:</strong> CSVV is usually associated with other vasculitis and connective tissue disorders and patients turning ANCA positive somewhere in the course of the disease is of ominous sign and hence, it becomes mandatory to keep these patients on a long term vigil. </p>


PEDIATRICS ◽  
1972 ◽  
Vol 50 (6) ◽  
pp. 940-953
Author(s):  
Jane Schaller ◽  
Ralph J. Wedgwood

Juvenile rheumatoid arthritis (JRA) is a disease of varying clinical manifestations, although all patients share the common property of arthritis. In following 124 children with JRA at a children's arthritis clinic (mean disease duration, 7.5 years), it was apparent that there were three distinct subgroups of disease distinguished by their clinical manifestations, prognosis for joint disability, types of extra-articular complications, and serologic findings. Systemic disease, characterized by high intermittent fevers and rheumatoid rash as well as other systemic manifestations, occurred in 32 of 124 patients (26%). All patients also had polyarthritis, generally beginning during the first six months of disease. More boys (18) than girls (14) were affected. No patient had iridocyclitis or positive tests for antinuclear antibodies or rheumatoid factor. Systemic symptoms alone were not a cause of permanent morbidity, but eight of 32 patients have incurred severe joint disability. Polyarticular disease unassociated with prominent systemic disease occurred in 46 of 124 patients (37%), predominantly girls (38 girls, eight boys). Multiple joints were involved, characteristically including small hand joints. Ten patients had antinuclear antibodies; six had positive latex agglutination tests for rheumatoid factor. Eight of 46 had severe joint disability. Persistent pauciarticular disease, characterized by arthritis limited to five or fewer joints, occurred in 46 of 124 patients (37%), predominantly girls (34 girls, 12 boys). Large joints were chiefly affected (knees, ankles, elbows); small hand joints were spared. Nine of 46 patients had antinuclear antibodies. No patient has had severe joint disability, but 12 of 46 have had iridocyclitis and ten have suffered some degree of permanent visual damage. Recognition of these disease patterns is useful in the diagnosis and care of children with JRA. Furthermore, these observations suggest that what we now call JRA may in fact be more than a single disease.


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