Fever in Rheumatological Diseases

What are rheumatological diseases ?

• Constellation of symptoms/sign, autoimmune markers, serological tests.

• Primary mimics are infection, malignancy, orthopedic and chronic pain conditions

• Diagnosis rest on clinics as there no single diagnostic test

• Exclusion of mimics are essential before starting treatment with corticosteroids and allied drugs.

• Fever is one of the commonest symptoms of pediatric rheumatological disease.

• The commonest causes of fever in pediatric population include infections, malignancies and non-infectious inflammatory diseases.

• It is important to rationally exclude the infections and malignancies before considering Non-infectious inflammatory (Rheumatological) diseases as the cause of fever.

Presentation of rheumatic diseases

• Joint pain• Fever• Rash• Fatigue• Organ system involvement (Muscles,

hepatic, renal, cardiopulmonary)

Identification of common mimics

Malignancy – Night time awaking, limb pain

Reactive arthritis – Oligoarticular, usually lower extremities, skin signs, fever, malaise and fatigue anorexia

Inflammatory bowel disease – Fever, weight loss, anorexia, erythema nodosum, pyoderma gangrenosum.

Hereditary periodic fever syndrome – Recurrent bouts of fever, arthritis pleural/peritoneal inflammation and skin rash.

Pediatric rheumatological conditions associated with fever• Systemic onset JIA (one of the commonest causes of fever

in pediatric rheumatology clinic)

• Vasculitis (large, medium and small vessel)

• Classical autoimmune diseases ( eg, SLE, Sjogren’s etc)

• Inflammatory myositis (Juvenile dermatomyositis, Polymyositis)

Pediatric rheumatological conditions associated with fever

• Miscellaneous autoimmune diseases (Sarcoidosis, Kikuchi, ALPS)

• Periodic fevers/Auto inflammatory syndromes (PFAPA, CAPS, TRAPS, FMF etc)

• Fever associated with immunosuppressed pediatric rheumatology patient

Fever in patients with arthritis

Edmonton criteria for Systemic onset JIA (SJIA)Arthritis in 1 or more joints + fever of at least 2 weeks' duration. Signs or symptoms must have been documented daily for at least 3 days and accompanied by 1 or more of the following:

• Evanescent rash, • Generalized lymphadenopathy, • Hepatosplenomegaly, • Serositis.

Evanescent rash of SJIA

Characteristic features of SJIA

• Arthritis might be delayed by weeks, months and years.

• Fever tends to be quotidian appearing at about the same time everyday.

• Child tends to be well in between fever spikes.

Macrophage activation syndrome

• The top fever chart is of a child with SJIA (Classical quotidian fever)

• The second fever chart is of a child with systemic onset JIA who has developed MAS with hectic fever.

(Dreaded complication of SJIA)

Macrophage activation syndrome

• Think of MAS if any patient presents with multi-organ dysfunction with hectic fever and is unresponsive to antibiotics and blood culture shows no growth.

• MAS can be the initial manifestation of SJIA in a patient.

• Clinical clue can be drop in ESR and blood counts with patient becoming sicker by the day.

• SLE is a prototype of autoimmune disease.

• Fever is seen in 35-90% of patients.

• Fever tends to be moderate to high grade.

• Suspicion is based on associated features as shown in pictures.

MALAR RASH

Systemic lupus erythematosus (SLE)

PALATAL ULCER OF SLE VASCULITIC RASH

Think of SLE when in an inflammatory condition ESR is high and CRP is normal

SLE

• Fever is seen in 16 to 65% of patients of JDM.

• Pattern recognition is important in all these diseases.

Gottron’s papule

Calcinosis cutis

Juvenile Dermatomyositis

Heliotrope rash and Gottron’s papules in JDM

Fever and vasculitis

• Vasculitides can be of predominant Large, medium or small vessel involvement.

• Fever can be the main presenting feature of many of these conditions.

• Kawasaki disease one of the commonest pediatric vasculitis and can be a prototype for fever with vasculitis.

Kawasaki Disease

Fever persisting at least 5 days with presence of at least 4 principal features:

1.Changes in Extremities :Erythema, edema of hands/feet or Periungual peeling of fingers, toes

2.Polymorphous Exanthem

3.Cervical Lymphadenopathy (> 1.5-cm diameter)

4.Changes in lips and oral cavity

5.Bilateral bulbar Conjunctival injection without exudate

Desquamation in a febrile child should arouse suspicion of KD

Polyarteritis nodosa presenting with febrile illness with digital ischemia and arthritis

Take home message

• Fever is an important symptom of pediatric rheumatological disease.

• Detailed history and a thorough physical exam leads to a diagnosis in most of these patients.

• Pattern recognition is important for diagnosis.