When you have eliminated the impossible, whatever remains, however improbable, must be the truth
I was asked to see a 32-year-old man for evaluation of severe right testicular pain of 2 years' duration.
Two years I saw the patient, he developed pain in both of his testicles—the right one more than the left one, with increasing pain despite narcotics. He was sent to an infectious disease specialist, who ruled out infection. He was treated with meloxicam, without improvement.
MRI of the pelvis showed that both vasa deferens were distended, with thickened walls and avid enhancement. There was focal rim enhancement and fluid collection along the vas deferens abutting the superolateral aspect of the urinary bladder, right more than left. Bilaterally, the seminal vesicles were diffusely thickened and atrophic. A peripheral zone of the prostate was suggestive of prostatitis. The findings were compatible with a chronic inflammatory process of the vasa deferens, seminal vesicles, and prostate, with focal abscess along the left vas deferens.
Surgery was performed to better define the cause and extent of the patient's pain and inflammation and to rule out infection or tumor. Pathology revealed chronic inflammation of the right vas deferens; vastitis nodosa; and inflammation and scarring of the prostate and seminal vesicles, chronic in nature. Cultures were negative, and no tumor was found.
The patient was treated with narcotics and oral methylprednisolone, but as soon as the steroid taper was completed, the pain returned. The patient was then treated with methylprednisolone twice daily with a slow taper over the next 2 weeks; he experienced marked improvement, but as soon as the steroids were stopped, his pain returned.
The patient denied any history of fever, night sweats, weight loss, joint inflammation, rash, neuropathy, sinus or lung problems, kidney disease, or clinical features consistent with an autoimmune disorder. All serologic tests were negative, including rheumatoid factor, antineutrophil antibody, and antineutrophil cytoplasmic antibody. Serum levels of immunoglobulin G4 (IgG4) were normal.
Clearly, the patient had a profound inflammatory process in his genitourinary area that was responsive to moderate- to high-dose oral steroids, but with return of inflammation and pain as soon as they were tapered. The patient received 500 mg of intravenous methylprednisolone followed by oral steroids—again with a flare of pain and inflammation.
The differential diagnosis included infection, autoimmune disorder, and vasculitis. These were ruled out on the basis of the clinical picture, blood tests, and pathology.
Given the steroid responsiveness and the combination of both scarring and inflammation pathologic examination, the pathology specimens were reevaluated, looking specifically for IgG4 disease characteristics. Tissue showed dense lymphoplasmacytic infiltrate, storiform (star-shaped) fibrosis, phlebitis, and increased numbers of IgG4-positive plasma cells.
Once IgG4 disease was diagnosed, the patient was treated with rituximab 1000 mg at baseline, achieving complete disease control 2 weeks later. His disease flared again 1 year later and again responded to rituximab.
As Sherlock Holmes said, "When you have eliminated the impossible, whatever remains, however improbable, must be the truth."
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Any views expressed above are the author's own and do not necessarily reflect the views of WebMD or Medscape.
Cite this: Severe Testicular Pain: Crack the Case - Medscape - Jan 03, 2017.