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The nailfold capillaroscopic (NC) patterns may be useful in the differential diagnosis of CTDs with RP. The NC patterns for SSc and PM/DM are both sensitive and specific to the diseases, while the SLE and MCTD patterns exhibit high specificity but relatively low sensitivity.

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Immunoglobulin G4-related disease (IgG4-RD) is a systemic autoimmune disease, primarily affecting various organ systems such as the pancreas, biliary tree, and liver. The orbit, salivary glands, the thyroid, facial skin, the trigeminal nerve, and the cervical lymph nodes are often affected by IgG4-RD in the head and neck region. 1 Although the ear is rarely affected, few case reports (we found 11 cases, listed in Table 1) of otologic manifestation can be found in the literature. 2-6 We excluded one case in Israel which was presented only in the congress. Of the 11 cases, seven were reported in Asia, and all the 11 cases were treated with steroids, one of them with a combination of methotrexate. Herein, we report the 12 th case of IgG4-RD involving the middle and inner ear. We treated this patient with cyclophosphamide and rituximab, which have never been used to treat otologic symptoms, but were used to treat other organs affected by IgG4-RD. 7-10 Pure tone audiometry was recorded serially to define the clinical improvement. In addition, we found that early treatment may lead to better outcomes.