Pediatric Granulomatous Arthritis (PGA) – aka Juvenile Systemic Granulomatosis, Blau syndrome, Early Onset Sarcoidosis, or Jabs Syndrome

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SAID group:
Granulomatous, Increased NF-kB Signalling

NOD2 (certain mutations)

Autosomal dominant.

Affects all races.

Unknown, but rare.

Timing of symptoms:
Intermittent or persistent daily fevers, rash and arthritis.

Age of onset:
Rash often develops by 4 months of age, fevers and other symptoms present by 4 years of age. [34]

Skin cutaneous:
First symptom: scaly plaques. The rash often starts on the face, then on the torso or chest. Biopsies with non-caseating granulomatous dermatitis. [34]

Intermittent-persistent daily fevers. Some have cranial neuropathies. 80% have vision damage and joint deformities if untreated. Some cases have peripheral nerves affected. [34]

Not noted. [34]

Uveitis (some with blindness). 50% with cataracts, 1:3 patients get secondary glaucoma. Inflamed conjunctiva (conjunctivitis), and/or inflammation of the lacrimal glands, retina and optic nerves is common. [34]

Some have atrial hypertension and/or pericarditis. Some cases with lung involvement. [34] [35]

Hepatomegaly, splenomegaly or hepatosplenomegaly. Some with abdominal pain, higher risk for kidney and/or liver issues. [34] [35] [36]

Splenomegaly, lymphadenopathy. [34] [35] [36]

Joints bones muscles cartilage:
Symmetrical chronic polyarthritis or oligoarthritis of the wrists, knees, ankles with a boggy appearance is usually caused by an exuberant tenosynovitis. [34] [35] [36]

Some with vasculitis, leukocytoclastic vasculitis. [34]

Not noted. [34]

Abnormal labs:
High CRP and ESR, angiotensin converting enzyme (ACE), immunoglobulins. Anemia, leukopenia, eosinophilia, hematuria, proteinuria, pyuria, abnormal liver function tests (LFTs). [34] [36]

Search Keywords:
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Photo credit:

Glass 2nd, D. A., Maender, J., & Metry, D. (2009). Two pediatric cases of Blau syndrome. Dermatol Online J, 15(5). Retrieved from


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