Case 2
A 73-year-old woman developed RA at the age of 69 years and had a history of transient ischemic attack at 67 years of age but no recurrence. She was prescribed NSAIDS and Chinese herbs by local physicians. She was hospitalized in our department in March 2021 for poor disease control. She complained of intermittent arthralgia, morning stiffness over 2 h daily, and fever, Tmax 38.5℃. Initial significant findings were tenderness and swelling of the metacarpophalangeal (MCP) joints of the left 2~5 digit, bilateral wrists, elbows, shoulders, and fine crackles in bilateral lower lung fields.
Initial laboratory findings showed that RF, CCP antibody, and ANA were positive (55.5 IU/mL, >200 U/mL, and 1:3200, respectively), while ENA and ANCA were negative. CRP and ESR increased to 30.3 mg/L and 50 mm/h, respectively. Markers of the tumor, HBV, and TB were negative. PFT showed normal ventilatory function and diffusion capacity. The X-ray revealed few erosions in both hands and wrists. Table 2 showed the patient’s laboratory findings during the hospitalization. Initial chest HRCT images showed a typical UIP pattern with marked honeycombing and traction bronchiectasis in anterior and subpleural upper lobes and left middle lobe, basal and subpleural right lower lobe, along with few gross glassy changes, sparing the central part of the lower lobes (Fig. 1 E, F, G, H, more details seen in Additional file 2 ). GGO and fibrosis were scored 5 and 10, respectively. Since the morphological pattern showed UIP with RA, the diagnosis was RA-UIP.
Regarding RA activity, DAS28-CRP scored 4.77 showing moderate disease activity. The fever was confirmed to be induced by the RA activity, excluding other conditions such as hypersensitivity pneumonia and pulmonary infections, and the treatment was initiated with 15 mg of prednisolone daily tapered by 2.5 mg every month, 5 mg of tofacitinib 2 times daily, and 25 mg of IGU 2 times daily.
She was referred again to assess the therapy 4 months later. The arthralgia improved gradually and the fever lessened. The prednisolone dose was 5 mg daily. Tofacitinib 10 mg daily plus IGU 50mg daily were maintained all the time. The chest HRCT demonstrated that lung lesions improved (Fig. 1 E1, F1, G1, H1 , more details seen inAdditional file 2 ). Laboratory findings, PFT, and HRCT scores improved (Table 2 ).
Nevertheless, in November 2021, she developed arthralgia continuously and coughed intermittently. Disease flares emerged when tofacitinib was discontinued for unavailablility in the local hospital 1 month after being discharged, though the prednisolone 5 mg daily plus IGU 50mg daily was maintained all the time. We observed increased CRP, ESR, worse PFT and HRCT scores (Table 2 ), and a progressive changes in the lower lobe of the lung on chest HRCT (Fig. 1 E2, F2, G2, H2 , more details seen in Additional file 2 ). Considering that RA and UIP relapses might result from the unavailable tofacitinib, she was resumed with 5mg of tofacitinib 2 times daily.
In May 2022, tofacitinib was discontinued again due to the local outbreak of COVID-19 2 months later. Only the prednisolone 5 mg daily was maintained all the time. Besides the recurring arthralgia and cough intermittently, swelling in face and limbs and myalgia in upper limbs emerged and made her unable to lift her arms over the shoulders. The disease seemed to flare again (Table 2 ). CRP increased up to12.4 mg/L, and a progressive shadow in the lower lobe of the lungs appeared on chest HRCT compared with the previous scan (Fig. 1 E3, F3, G3, H3 , more details seen in Additional file 2 ). Every time, a rapid arthritis control was obtained with the reintroduction of tofacitinib and IGU. She continued with 5 mg of prednisolone daily, 5mg of tofacitinib 2 times daily, and 25mg of IGU 2 times daily and was prescribed enough for 3 months. So far, she is still being followed-up with and remains in remission (Table 2 ) of RA-UIP with the continued use of tofacitinib plus IGU.