Sjogren’s Syndrome with Multiple Bullae and Pulmonary Nodular Amyloidosis: Discussion

18 Feb
2015

The nodular lesions in the biopsy materials consisted of homo geneous material without internal structures surrounded by inflammatory mononuclear cells. They displayed the green birefringence characteristic of amyloid when viewed under polarized light after Congo red staining (Fig 4). As the plasma cells in the vicinity of amyloid deposits showed strongly positive to the anti-kappa chain antibody by immunoperoxidase staining, they might be thought of as localized monoclonal proliferation, but the plasma cells in the bronchiolar wall yield polyclonal immunoglobulin staining. The biopsy specimens from lip, stomach, rectum, bone marrow, and bronchus did not contain amyloid deposits. The bone marrow samples showed no evidence of proliferation and atypism of plas-macytes or lymphocytes. The diagnosis was nodular pulmonary amyloidosis. We administered prednisolone 40 mg/day for the management of dyspnea and the patient could have a symptomatic relief. However, the bullae did not disappear or diminish in size. canadian neightbor pharmacy

Discussion
Although pulmonary complications associated with Sjogren’s syndrome have attracted attention in recent years, bullae formation has been reported in only one case; pulmonary nodular amyloidosis is also a rare concomitant. Thus, this is the first report of a patient having these two complications.
In this patient, the primary and most interesting change was the infiltration of lymphocytes and plasma cells into the bronchiolar wall, without involvement of other lung regions. Extensive infiltration by inflammatory cells induced prominent immunoglobulin production and resulted in amyloid deposition. Monoclonal immunoglobulin staining of the plasma cells in the vicinity of amyloid deposits may be classified as plasma cell dyscrasia. Also, airway restriction by mononuclear cell infiltration caused check-valve mechanism, and the formation of multiple bullae. Small airway disturbances occur frequently in patients with Sjogrens syndrome, but bullae formation is a rare complication.

Figure 4. The nodular lesion showed green birefringence when viewed under polarized light after Congo red staining. This finding is characteristic of amyloid deposition (Congo red staining, original magnification X 80).

Figure 4. The nodular lesion showed green birefringence when viewed under polarized light after Congo red staining. This finding is characteristic of amyloid deposition (Congo red staining, original magnification X 80).

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