A 70-year-old woman is seen by her physician for evaluation of severe headaches. She noted these several weeks ago, and they have been getting worse. Although she has not had any visual aura but she has been intermittently losing vision in her left eye for the last few days. She denies new weakness or numbness, but she does reports jaw pain with eating. Her past medical history includes coronary artery disease requiring a bypass grafting 10 years prior, diabetes mellitus, hyperlipidaemia, and mild depression. Full review of symptoms is notable for night sweats and mild low back pain particularly prominent in the morning. Which of the following is the next most appropriate step?
Uveo-parotitis is seen in
Characteristic feature of SLE is -
Arthritis mutilans is due to:
In rheumatoid arthritis, the characteristic joint involvement is:
Which of the following conditions results in arthritis without erosion of the bones?
An elderly male presents with pain in his shoulders and hips. Temporal arteries are tender to palpation. ESR is 105 mm/L.
What are the extra-articular manifestations of RA?
True statements about ankylosing spondylitis include all except: a) HLA-B27 is found in 90% of sufferers b) Uveitis is found in 15 to 20% of sufferers c) The condition is more common in females d) Radiological changes can occur in the spine before symptoms
35 year old female patient complains of dry mouth and scratchy feeling in eyes. She is found to have antibodies against anti- SSA/Ro. Which of the following can be the most accurate sole criterion to diagnose the condition
Explanation: ### Immediate initiation of glucocorticoids - The patient's symptoms, including **severe headaches**, **transient vision loss (amaurosis fugax)**, **jaw claudication**, and **systemic symptoms** like night sweats in an elderly woman, are highly suggestive of **giant cell arteritis (GCA)**. [1] - **Prompt initiation of high-dose glucocorticoids** is crucial to prevent irreversible vision loss, which is a devastating complication of GCA. [1] *Aspirin 975 mg orally daily* - While aspirin may be considered as an adjunct in treating GCA to reduce thrombotic complications, it is **not the primary or immediate treatment** for the disease itself. - **High-dose aspirin** is typically not used for GCA and would not address the underlying inflammation causing ocular ischemia. *Measurement of erythrocyte sedimentation rate* - An **elevated ESR** is characteristic of GCA and can support the diagnosis. [1] - However, performing this test first would **delay crucial treatment** for GCA, potentially leading to permanent vision loss. [1] *Referral for temporal artery biopsy* - A **temporal artery biopsy** is the gold standard for confirming the diagnosis of GCA but should **not delay the initiation of glucocorticoids**. [1] - Treatment should begin immediately based on clinical suspicion, and the biopsy can be performed safely within **1-2 weeks of starting steroids** without significantly impacting diagnostic yield. [1]
Explanation: ***Sarcoidosis*** - **Uveo-parotitis** (also known as Heerfordt's syndrome or uveoparotid fever) is a rare manifestation of **sarcoidosis**, characterized by swelling of the **parotid glands**, anterior **uveitis**, fever, and sometimes facial nerve palsy [1]. - Sarcoidosis is a chronic inflammatory disease that can affect multiple organs, with **granuloma formation** being its pathological hallmark [1]. *Mumps* - Mumps primarily causes **parotitis**, an inflammation of the parotid glands, but it is typically not associated with **uveitis**. - It is a viral infection characterized by sudden onset of fever, headache, malaise, myalgia, and swelling of one or both parotid glands. *Measles* - Measles is a viral infection that causes a **generalized maculopapular rash**, fever, cough, coryza, and conjunctivitis, but it does not cause **uveo-parotitis**. - Ocular involvement in measles is usually limited to **conjunctivitis** rather than deeper inflammatory conditions like uveitis. *Cystic fibrosis* - **Cystic fibrosis** is a genetic disorder affecting exocrine glands, leading to thick mucus production in the lungs, pancreas, and other organs. - While it can lead to various systemic complications, it is not associated with **uveo-parotitis**.
Explanation: ***Polyserositis*** - **Polyserositis**, including **pleuritis** and **pericarditis**, is a common and characteristic manifestation of **Systemic Lupus Erythematosus (SLE)**, affecting a significant portion of patients [1]. - Inflammation of serous membranes leads to chest pain, effusions, and effacement of normal tissue planes [1]. *Cavitating lesion in lung* - **Cavitating lung lesions** are generally not a characteristic feature of SLE; they are more commonly associated with infections (e.g., tuberculosis, fungal infections), vasculitis (e.g., granulomatosis with polyangiitis), or malignancies. - While SLE can cause pulmonary manifestations like **lupus pneumonitis** or **diffuse alveolar hemorrhage**, these typically do not present as cavitating lesions [1]. *Joint deformity* - Although **arthralgias** and **arthritis** are very common in SLE, leading to pain and swelling, **non-erosive arthritis** is the hallmark. - Significant **joint deformities** (e.g., erosions, subluxations) are rare in SLE and are more characteristic of conditions like **rheumatoid arthritis** (with its destructive synovitis). *Uveitis* - **Uveitis** (inflammation of the uvea of the eye) is not a common or characteristic manifestation of SLE. - It is more typically associated with other systemic inflammatory conditions such as **spondyloarthropathies** (e.g., ankylosing spondylitis), **sarcoidosis**, or **Behcet's disease**.
Explanation: ***Psoriatic arthritis*** - **Arthritis mutilans** is a severe and destructive form of **psoriatic arthritis** characterized by rapid bone resorption and telescoping digits [1]. - It results in significant **deformity** and functional impairment, particularly in the hands and feet [1]. *Rheumatoid arthritis* - While rheumatoid arthritis can cause severe joint destruction, it typically presents with **symmetrical polyarthritis** and joint erosions, but not the specific "telescoping digit" deformity seen in arthritis mutilans. - It more commonly leads to deformities like **ulnar deviation** and **swan-neck deformities**. *Osteoarthritis* - Osteoarthritis is a degenerative joint disease characterized by **cartilage loss** and **osteophyte formation**, primarily affecting weight-bearing joints. - It does not cause the rapid bone resorption or inflammatory damage characteristic of arthritis mutilans. *Rheumatic arthritis* - This term is ambiguous; if referring to **rheumatic fever arthritis**, it is a migratory polyarthritis following a Group A Streep infection and is transient, not leading to chronic destructive deformities like arthritis mutilans. - If referring to a general inflammatory arthritis, it lacks the specificity for diagnosing arthritis mutilans.
Explanation: ***Metacarpophalangeal joint*** - **Rheumatoid arthritis** classically affects the **small joints** of the hands and feet in a **symmetrical** pattern [1]. - The **metacarpophalangeal (MCP) joints** are among the most frequently involved, leading to pain, swelling, and characteristic deformities [1]. *Hip joint* - While **hip involvement** can occur in late-stage or severe rheumatoid arthritis, it is not considered the **characteristic initial presentation** of the disease. - Initial symptoms usually present in smaller, more peripheral joints. *Spine* - **Spinal involvement** in rheumatoid arthritis primarily affects the **cervical spine**, particularly the atlantoaxial joint, which can lead to **subluxation** [1]. - However, the lumbar and thoracic spine are typically spared, and it is not the initial characteristic joint involvement [1]. *Knee* - The **knee joint** can be affected in rheumatoid arthritis, often presenting with **synovitis and effusion**. - It is a **larger joint** and while common, it is not as characteristic of the initial defining joint involvement as the MCP joints [1].
Explanation: ***SLE*** - **Systemic lupus erythematosus (SLE)** arthritis is typically **non-erosive**, meaning it usually doesn't cause damage to the bone or cartilage, though it can cause joint deformities like **Jaccoud's arthropathy** [1]. - The inflammation in SLE primarily affects the **synovium** and surrounding soft tissues without directly eroding the articular surfaces [1]. *Rheumatoid arthritis* - **Rheumatoid arthritis (RA)** is characterized by **erosive arthritis**, where chronic inflammation of the synovium leads to destruction of articular cartilage and **subchondral bone erosions**. - These erosions are visible on **radiographs** and are a hallmark of the disease progression. *Psoriasis* - **Psoriatic arthritis** can be **erosive**, especially in its more severe forms, leading to **"pencil-in-cup" deformities** and destruction of joint structures [1]. - While some forms may be less aggressive, significant **bone erosion** is a recognized feature [1]. *Osteoarthritis* - **Osteoarthritis** is characterized by the **degeneration of cartilage** and subsequent changes in subchondral bone, including **osteophyte formation** and **subchondral sclerosis**, rather than direct erosion. - While it involves structural changes to the bone and cartilage, it's not typically described as an erosive arthritis in the same sense as inflammatory arthropathies, but rather a degenerative process that leads to **cartilage loss** and **bone remodeling**.
Explanation: ***Temporal arteritis*** - This condition presents with **shoulder and hip pain (polymyalgia rheumatica-like symptoms)**, **tender temporal arteries**, and a **markedly elevated ESR**, all classic features of giant cell arteritis (temporal arteritis). - It is a **large vessel vasculitis** predominantly affecting individuals over 50 years of age and can lead to irreversible **blindness** if not treated promptly. [1] *Granulomatosis with polyangiitis (Wegener granulomatosis)* - Primarily affects the **upper and lower respiratory tracts** and **kidneys**, often presenting with chronic sinusitis, lung nodules, and glomerulonephritis. [1] - While it can cause an elevated ESR, the primary symptoms described (shoulder/hip pain, tender temporal arteries) are not typical of this condition. *Cryoglobulinemic vasculitis* - This vasculitis is often associated with **hepatitis C infection** and typically presents with **palpable purpura**, **arthralgias**, and **glomerulonephritis**, none of which are described. - It primarily involves **small to medium-sized vessels** and does not typically manifest with tender temporal arteries or polymyalgia rheumatica. *Churg-Strauss syndrome* - This condition, now known as **eosinophilic granulomatosis with polyangiitis**, is characterized by **asthma**, **eosinophilia**, and **multi-organ involvement**. [2] - While it is a systemic vasculitis and can cause musculoskeletal pain, the specific presentation of tender temporal arteries and polymyalgia rheumatica is not its hallmark. [2]
Explanation: ***All of the options*** - **Rheumatoid arthritis (RA)** is a systemic inflammatory disease that can affect multiple organs beyond the joints [1]. Therefore, **pericarditis**, **pulmonary fibrosis**, and **scleritis** are all recognized extra-articular manifestations [2]. - The presence of various extra-articular features often indicates more severe or long-standing disease and is associated with increased morbidity and mortality in RA patients. *Pericarditis* - **Pericarditis** is a recognized cardiovascular manifestation of RA, involving inflammation of the **pericardium**. - While many cases are asymptomatic, it can cause chest pain and, rarely, **pericardial effusion** or **constrictive pericarditis**. *Pulmonary fibrosis* - **Interstitial lung disease (ILD)**, including **pulmonary fibrosis**, is a significant pulmonary complication of RA [2]. - It can lead to progressive shortness of breath, cough, and is a major cause of morbidity and mortality in RA patients. *Scleritis* - **Scleritis** is an ocular manifestation of RA, presenting as severe inflammation of the sclera (the white outer layer of the eyeball) [1]. - It can cause severe pain, redness, and vision changes, and requires prompt treatment to prevent visual impairment [1].
Explanation: ***BCD*** - This option correctly identifies the statement "The condition is more common in females" as false, making it an **incorrect statement** about ankylosing spondylitis. Also, the statement "Radiological changes can occur in the spine before symptoms" is true. This option is consistent with the prompt to find all *except*. - Ankylosing spondylitis is significantly **more common in males** than females, with a male-to-female ratio of about 2-3:1, thus making the original statement (c) false. *AC* - This option incorrectly states that "HLA-B27 is found in 90% of sufferers" is false. **HLA-B27** is indeed found in about 90-95% of individuals with ankylosing spondylitis, making statement (a) true [1]. - This option also incorrectly states that the condition being more common in females is true, when it is actually **more common in males**. *ACD* - This option incorrectly includes statement (a) as false; **HLA-B27** is found in the vast majority of patients with ankylosing spondylitis [1]. - It also incorrectly implies that statements (c) and (d) are false when (c) is false (making the condition more common in females an incorrect statement) and (d) is true. **Radiographic changes** such as sacroiliitis can precede overt symptoms in some patients [1]. *BD* - This option incorrectly implies that "Uveitis is found in 15 to 20% of sufferers" is false [1]. **Acute anterior uveitis** is a common extra-articular manifestation, occurring in 25-40% of patients with ankylosing spondylitis. - It also includes that "Radiological changes can occur in the spine before symptoms" is false, which is incorrect. **Subclinical inflammation** and early radiological changes often precede the development of clinical symptoms [1].
Explanation: Lip tissue biopsy - A lip biopsy demonstrating focal lymphocytic sialadenitis in the minor salivary glands is a gold standard for diagnosing Sjogren's syndrome, especially when other criteria are met. - This pathological finding provides definitive evidence of the characteristic autoimmune inflammation affecting the exocrine glands. Objective measurement of lacrimal and salivary gland secretion - While important for evaluating the severity of dryness (e.g., Schirmer's test for eyes, sialometry for saliva), these are functional measurements and not a sole diagnostic criterion. - They can be affected by other factors and do not directly confirm the autoimmune pathology like a biopsy does. Lymph node biopsy - A lymph node biopsy is not a standard diagnostic procedure for Sjogren's syndrome. - It might be performed if lymphoma is suspected, as Sjogren's patients have an increased risk, but it's not used for initial diagnosis of the syndrome itself. Positive rheumatoid factor - A positive rheumatoid factor (RF) is common in Sjogren's syndrome (present in 75-90% of patients), but it is not specific and can be elevated in other autoimmune diseases (e.g., rheumatoid arthritis) or even in healthy individuals. - Therefore, it cannot be used as a sole diagnostic criterion. Antibodies to Ro (SSA) are characteristic of the condition and are often found in association with anti-La antibodies [1].
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