A rare deformity and bilateral concurrent patellar tendon and quadriceps tendon ruptures in a patient with relapsing polychondritis a case report

İngilizce (OpenAlex)

Dear Editor, Relapsing polychondritis (RP) is an episodic autoimmune disease that is associated with inflammation of cartilaginous structures.1 RP affects elastic cartilage of the ears and nose, hyaline cartilage of peripheral joints, fibrocartilage of axial sites and cartilage of tracheobronchial tissues. Arthritis generally occurs as episodic, migratory, nonerosive, seronegative and asymmetric oligoarthritis or polyarthritis.2 No deformity is seen in the joints of RP patients. However, development of some deformities and erosions in the joint was observed in RP patients who had rheumatoid arthritis (RA).3, 4 To our knowledge, deformity in the joints of an RP patient who has no other rheumatologic or autoimmune disease has not previously been reported. We herewith present a patient with RP who had developed a deforming arthritis and bilateral patellar and quadriceps tendon ruptures. A 19-year-old woman sought medical advice for complaints such as swelling in her left knee and arthralgia in both knees and in her fingers. In her anamnesis, it was determined that her complaints had begun 6 or 7 years earlier, with swelling and pain in the fingers, lasting for 1–2 days. During this undiagnosed period, the patient had used several nonsteroidal anti-inflammatory drugs (NSAIDs) and glucocorticoids. Four years previously, she had experienced swelling and arthralgia in her knees, lasting for 1 or 2 days. Her complaints had continued and, 1 year previous to us seeing her, she had been prediagnosed with RA and treated with methotrexate (MTX) 10 mg/week and prednisolone 5 mg/day. However, the patient had stopped MTX treatment after 2 months. Six months before she sought our advice, a red lesion had occurred on her right leg. Because of itching, this lesion had grown into 10–15 lesions. Lesion healing was characterized by scabbing. Then, 3 months prior to coming to our clinic, her hand had developed deformity, although she experienced no pain. She also reported coughing and tinnitus. Her physical examination at our clinic revealed telengiectases on the malar regions, as well as boutonniere deformities on the bilateral fingers (Fig. 1a). Hyperpigmented lesions on her right leg were diagnosed as impetigo. Bilateral shoulder, knee, wrist and metacarpophalangeal (MCP) joint motions were painful. The patient also had swelling on the left knee, but could walk with assistance. In her laboratory examination, the following values were determined: erythrocyte sedimentation rate, 39 mm/h; C-reactive protein, 10.1 mg/dL (normal, 0.0–0.5 mg/dL); and hemoglobin, 12.6 g/dL. The values of serum complement, rheumatoid factor, anti-cyclic citrullinated protein, anti-nuclear antibodies anti-double stranded DNA, and other autoantibodies were normal. Hand radiographs were unremarkable, except for the deformities (Fig. 1b). Magnetic resonance imaging (MRI) revealed bilateral patellar and quadriceps tendon ruptures at the knees (Fig. 2). Also, the patient reported that she had a slight fall in her home 3 months earlier, which had resulted in swelling and arthralgia in her left knee, lasting 1 or 2 days. During this time, she had developed swelling on her right pinna, along with erythema, warmth and tenderness, but sparing the non-cartilaginous lobe. Two days later, her left ear had shown similar developments. The patient also reported that her first ear involvement had been 3 years earlier and that, for the past year, these ear complaints had occurred every month. These symptoms suggested RP. Although her auditory examination was normal, multislice computed tomography scan and cardiac MRI examinations showed dilatation on the trachea and laryngopharyngeal examination revealed a hyperemic lesion on the epiglottis. In addition, Staphylococcus aureus was identified in the nose secretion. However, no other rheumatological disease was found. Based on these clinical and laboratory examinations, RP was diagnosed according to the criteria of McAdam.5 The patient was treated with MTX (25 mg/week) and prednisolone (250 mg/day pulse treatment over 3 days, then 15 mg/day). After clinical improvement, she was referred to the orthopedics and traumatology clinic for repair of tendons. Relapsing polychondritis affects cartilaginous structures and proteoglycan-rich tissues. Auricular chondritis, involving the cartilaginous portion of the pinna, develops in almost all patients who have RP.2 Inflammation is generally bilateral and leads to pain, redness, warmth, swelling and tenderness on the cartilaginous portion of the ears, while sparing the non-cartilaginous lobe.1 Acute inflammatory episodes last for a few days or weeks in the majority of patients. Cartilage destruction, due to recurrent inflammatory episodes, may lead to flabby or droopy ears, or result in cauliflower ear.1, 2 Also, sensorineural hearing loss and vestibular dysfunction may occur because of inflammation in the middle ear and other audiovestibular structures.2 Arthritis is the second most common manifestation.6 It may occur in 50% to 85% of RP cases, and is the initial presenting symptom in 33% of RP patients.3, 6 The most common areas affected with arthritis are the costochondral junctions, MCP, proximal interphalangeal (PIP) joints, knees and wrists. Interestingly, while inflammation through RP results in extra-articular cartilage destruction in the ears and nose, it does not cause erosion on articular cartilage of RP patients who have arthritis.2 Rheumatologic or autoimmune diseases, such as RA, systemic lupus erythematosus (SLE), Sjögren's syndrome and spondyloarthropathies have been reported in 30% of patients with RP.1 Also, 30% of RP patients may have erosive arthritis, which is associated with RA.3, 4 In our case, deformities were observed in the PIP and distal interphalangeal (DIP) joints of both hands. However, there was no erosion on the joints that had arthritis. In addition, bilateral rupture of patellar and quadriceps tendons, which are the strongest tendons of the knee joint, occurred with minimal trauma. There was fibrocartilage tissue on the proximal and distal attachment regions of the patellar tendon,7 the most common areas for tendon rupture.8 The bilateral rupture of patellar and quadriceps tendons indicates that inflammation of articular cartilage and insertion of tendons might have caused a susceptibility to rupture in our case. Laryngo-tracheobronchial disease occurs on about 50% of patients with RP.3 Inflammation and damage of laryngeal, epiglottal, tracheal and bronchial cartilages cause dyspnea, a non-productive, persistent cough, wheezing and inspiratory stridor.2 Our case showed redness on the epiglottis, persistent cough and dilatation on the trachea. In conclusion, even though erosions do not develop in RP arthritis, deformity can be observed in rare cases. In addition, the ruptures may easily occur as the result of minimal trauma, even on the strongest tendons.