The heart is the most commonly involved organ and cardiac involvement is associated with adverse prognosis.
7, 8 In comparison, ANA-negative patients with polyarticular disease tend to present older with a cumulative, symmetrical arthritis, and with a different pattern of joint involvement that more commonly involves the hips and shoulders.
The organ that is most commonly involved in IgG4-related sclerosing disease is the pancreas; such involvement is termed autoimmune pancreatitis (AIP) [1].
Although the oculomotor is the commonly involved nerve in OM, there are case reports for the involvement of the 4th and 6th cranial nerves.
In addition to the kidney, the most commonly involved organ was the intestinal tract (55.9%), followed by cardiac involvement (46.7%).
The most commonly involved elbow was the right (52.8 %), and 75%% of the cases did not have ulnar nerve involvement.
Commonly hip involvement at presentation.
The axonopathic effect is characterized by dystrophic changes in the distal axons of spinal proprioceptive tracts with little or more commonly no involvement of motor neurons [ 19, 27].
Evidence from brain metabolism studies suggests that EOAD is associated with changes that are more extensive, and studies most commonly report involvement of the precuneus and occipital cortex [ 24- 26], and one study reports extension to the frontal cortex and subcortical grey matter [ 26].
In patients with cerebral edema secondary to OTC deficiency, MRI commonly shows symmetric involvement of both cerebral hemispheres and pallidal nuclei, with specific involvement of the insula, frontal, and temporal lobes, and sparing the brainstem and cerebellum.
