An 18-year-old girl with history of chronic sinusitis and repeated childhood chest infections was referred to our hospital for work up of a nonresolving pneumonia.
The patient was first seen and evaluated in Pulmonology Clinic. She had a chronic productive cough but no fever, weight loss, or hemoptysis. Chest X-ray revealed a residual right middle lobe infiltrate with no pleural effusion. Computed tomography (CT) scan of the chest revealed patchy airspace opacities in the right lower lobe associated with bilateral lower lobe bronchiectatic changes. Interestingly, there were multiple structural abnormalities incidentally found in the form of an abdominal situs ambiguous with the liver seen in the midline, a right-sided stomach, and multiple small splenules in the right upper abdomen (Figure 1). Bronchoscopy with bronchoalveolar lavage was positive for Haemophilus influenzae. All other differentials including Mycobacterium tuberculosis, fungal infections, and malignancy were ruled out.
During that same period, the patient gave history of two new complaints: joint pain and swelling and amenorrhea. Regarding her joint complaints, the patient gave history typical of RA with symmetrical joint pain and swelling involving her small and large joints with early morning stiffness of more than an hour. On examination her tender joint count (TJC) was 10 and swollen joint count (SJC) was 7. She had a strongly positive Rheumatoid Factor of 350 and an anti-cyclic citrullinated peptide (Anti-CCP) of 133. Her erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were mildly elevated at 21 and 5.27 mg/L, respectively. The rest of her autoimmune panel including ANA and ENA profiles was negative. X-rays of the hands, elbows, shoulders, knees, and feet bilaterally revealed maintained joint spaces and no evidence of erosions. Based on these findings the patient was diagnosed to have RA. Her calculated DAS-28 score was 5.77, her Simple Disease Activity Index (SDAI) 32.5, and her Clinical Disease Activity Index (CDAI) 32, all reflecting high disease activity. She was started on Methotrexate at a dose of 12.5 mg orally once weekly with supplemental folate. She was also kept on Prednisolone 10 mg orally daily with calcium and vitamin D.
As for her amenorrhea symptoms, the patient had irregular periods after menarche but became completely amenorrheic around 8 months prior to presentation. She had been married for one and a half years, had no children, and was not using any method of contraception. She was referred to Obstetrics and Gynecology Team for further evaluation and her work-up revealed the presence of a very high prolactin level of 4425 mug/L (normal range: 3-24 mug/L). another hormonal panel was within normal. She had no symptoms of galactorrhea, was not taking any new drugs, and had no history of renal, thyroid, or liver disease. She had no visual symptoms or headache or vomiting. Pelvic ultrasound showed a retroverted uterus with no abnormalities. She was given an appointment to do pituitary magnetic resonance imaging (MRI) in addition to Endocrinology followup.
Due to the earlier CT findings of situs ambiguous and history of chronic bronchiectasis, the diagnosis of KS was entertained. CT scan of the paranasal sinuses was therefore requested and it confirmed the presence of pansinusitis with complete opacification of the maxillary, sphenoid, and ethmoid sinuses (Figure 2). Based on the presence of this triad, she was diagnosed with KS and was admitted for full work-up and treatment on the 20th of October 2014. Magnetic resonance imaging (MRI) of the pituitary gland was done and it revealed a 2.2 x 2.1 x 1.2 cm intrasellar pituitary macroadenoma (Figure 3). She was seen and evaluated by Endocrinology Team and started on Cabergoline 0.5 mg once weekly and discharged in a stable condition with outpatient followup.
When the patient presented to Rheumatology Clinic the following month, she reported almost 80% improvement of her symptoms with significant reduction in her joint pain and complete resolution of morning stiffness and fatigue. Based on assessment that day, her DAS-28 score was 1.7 and her SDAI and CDAI scores were 4.9 and 3, respectively. Her prolactin level dropped to 293 mug/L. The patient achieved clinical remission and her Methotrexate dose was increased to 15 mg orally once weekly. She was instructed to taper down her steroids until tapering off. During the next visit three months later, she was still in remission with a DAS-28 score of 1.4, SDAI of 2.5, and CDAI of 2. Her Methotrexate dose was maximized to 20 mg once weekly. Repeated MRI of the pituitary gland revealed regression in the size of the macroadenoma to 1.4 x 1.4 cm with a further drop in her prolactin level to 28.9 mug/L. Cabergoline was increased to 1.5 mg orally twice a week on subsequent Endocrinology followup.