Rheumatology and Immunology Practice Questions

Q: Which of the following is FALSE about SAPHO syndrome?

The 'P' in SAPHO stands for psoriasis. SAPHO syndrome is a rare chronic inflammatory disorder that primarily affects the skin and the musculoskeletal system. It belongs to the group of Seronegative Spondyloarthropathies [1]. ### Explanation of the Correct Answer Option B is FALSE because the 'P' in SAPHO stands for Pustulosis, not Psoriasis. While psoriasis can be associated with the syndrome, the acronym specifically refers to the characteristic dermatological finding of Palmoplantar Pustulosis or Acne (specifically Acne conglobata or Acne fulminans). The full acronym stands for: * S: Synovitis * A: Acne * P: Pustulosis * H: Hyperostosis (excessive bone growth, typically of the sternoclavicular joint) * O: Osteitis (inflammation of the bone) ### Analysis of Other Options * Option A (RF Negative): As a member of the seronegative spondyloarthropathy family, patients are typically negative for Rheumatoid Factor. * Option C (HLA B-27 Positive): There is a known association with HLA B-27, though the prevalence (approx. 15-30%) is lower than in Ankylosing Spondylitis [1]. * Option D (Synovitis): This is a core component of the syndrome, often manifesting as inflammation of the large joints or the axial skeleton [1]. ### High-Yield Clinical Pearls for NEET-PG * Classic Site: The Anterior Chest Wall (sternoclavicular and manubriosternal joints) is the most common site of involvement, often showing a characteristic "Bull's Head" appearance on a technetium-99m bone scan. * Radiology: Look for Hyperostosis (thickening of cortical bone) and osteosclerosis. * Treatment: First-line therapy includes NSAIDs. For refractory cases, Bisphosphonates (due to their anti-inflammatory effect on bone) and TNF-alpha inhibitors are used.

Q: Pulseless disease is also known as which of the following conditions?

Takayasu arteritis. **Explanation:** **Takayasu Arteritis (TA)** is known as **"Pulseless Disease"** because it is a chronic, large-vessel vasculitis that primarily involves the aorta and its main branches [1]. The underlying pathology involves granulomatous inflammation leading to stenosis, occlusion, or aneurysmal dilation of the vessels. When the subclavian or axillary arteries are involved, it results in diminished or absent peripheral pulses (typically in the upper limbs) and a significant blood pressure discrepancy between the arms. **Analysis of Incorrect Options:** * **Giant Cell Arteritis (GCA):** Also a large-vessel vasculitis, but it primarily affects the extracranial branches of the carotid artery (e.g., temporal artery). While it shares histological features with TA, it occurs in patients >50 years and rarely presents with generalized pulselessness. * **Kawasaki Disease:** A medium-vessel vasculitis seen in children [1]. It is characterized by "mucocutaneous lymph node syndrome" and carries a high risk of coronary artery aneurysms, not large-vessel occlusion. * **Polyarteritis Nodosa (PAN):** A medium-vessel vasculitis that affects renal and visceral arteries, sparing the lungs. It typically presents with livedo reticularis, mononeuritis multiplex, and microaneurysms (beading on angiography), rather than loss of peripheral pulses. **Clinical Pearls for NEET-PG:** * **Demographics:** Most common in young females (<40 years), often of Asian descent. * **Gold Standard Diagnosis:** Conventional Angiography (shows "string of pearls" or tapered narrowing). * **Classification:** It is a Large Vessel Vasculitis (along with GCA) [1]. * **Clinical Sign:** Bruits (especially over the subclavian artery or aorta) and hypertension (due to renal artery stenosis).

Q: A 50-year old female presents with sudden blindness. There is scalp tenderness and pain while combing her hair. Temporal artery biopsy shows intimal thickening with granulomatous inflammation. What is the diagnosis?

Giant cell arteritis. ### Explanation **Correct Option: A. Giant cell arteritis (GCA)** The clinical triad of **sudden blindness** (due to anterior ischemic optic neuropathy), **scalp tenderness** (pain while combing hair), and **jaw claudication** in an elderly patient is classic for Giant Cell Arteritis (Temporal Arteritis). The histopathological hallmark is **granulomatous inflammation** [1] of the media with **intimal thickening** and fragmentation of the internal elastic lamina. It primarily affects large and medium-sized arteries, particularly the branches of the external carotid artery. **Why other options are incorrect:** * **B. Takayasu Arteritis:** While also a large-vessel vasculitis [2] with granulomatous inflammation, it typically affects females **under 40 years** ("young female arteritis") and involves the aorta and its primary branches, leading to absent pulses (pulseless disease). * **C. Polyarteritis Nodosa (PAN):** A medium-vessel vasculitis associated with Hepatitis B. It causes necrotizing inflammation (not granulomatous) and typically presents with renal failure, hypertension, and abdominal pain, sparing the lungs. * **D. Microscopic Polyangiitis (MPA):** A small-vessel vasculitis associated with p-ANCA. It presents with necrotizing glomerulonephritis and pulmonary capillaritis, lacking the granulomatous features seen in GCA. **NEET-PG High-Yield Pearls:** * **Association:** Strongly associated with **Polymyalgia Rheumatica (PMR)** (proximal muscle pain/stiffness) [1]. * **Diagnosis:** Elevated **ESR** (often >100 mm/hr) is a highly sensitive screening tool [1]. * **Biopsy:** Temporal artery biopsy is the gold standard [1], but "skip lesions" can occur; hence, a long segment (2-3 cm) should be sampled. * **Management:** Do not wait for biopsy results. Start **high-dose corticosteroids** immediately to prevent permanent blindness in the contralateral eye [1].

Q: All the following features are found in Sjogren syndrome, EXCEPT?

Granulomas in salivary glands. **Explanation:** **Sjögren Syndrome (SS)** is a chronic autoimmune disorder characterized by lymphocytic infiltration of exocrine glands, primarily the lacrimal and salivary glands. 1. **Why "Granulomas in salivary glands" is the correct answer:** The hallmark histopathological finding in Sjögren syndrome is **focal lymphocytic infiltration** (specifically CD4+ T cells and B cells), not granuloma formation. The presence of non-caseating granulomas in salivary glands is characteristic of **Sarcoidosis** (Heerfordt syndrome), which is a major differential diagnosis for sicca symptoms but is pathologically distinct from SS. 2. **Analysis of Incorrect Options:** * **Xerophthalmia (Dry eyes):** A cardinal feature caused by lymphocytic destruction of lacrimal glands, leading to keratoconjunctivitis sicca. * **Xerostomia (Dry mouth):** A cardinal feature resulting from decreased salivary flow due to glandular infiltration. * **Lymphoma:** Patients with Sjögren syndrome have a **40-fold increased risk** of developing B-cell lymphomas (most commonly MALT lymphoma). Persistent parotid swelling and a drop in rheumatoid factor levels are clinical red flags for malignant transformation. **High-Yield Clinical Pearls for NEET-PG:** * **Antibodies:** Anti-Ro (SS-A) and Anti-La (SS-B) are the most specific markers. * **Schirmer’s Test:** Used to quantify tear production (Positive if <5 mm in 5 minutes). * **Diagnosis:** Minor salivary gland biopsy (usually from the lip) showing a "Focus Score" ≥1 (aggregate of 50 or more lymphocytes). * **Extraglandular manifestations:** Raynaud’s phenomenon, cutaneous vasculitis, and interstitial lung disease (ILD).

Q: Multiple pulp stones are seen in which condition?

Ehlers Danlos syndrome. **Explanation:** **1. Why Ehlers-Danlos Syndrome (EDS) is correct:** Ehlers-Danlos Syndrome is a heterogeneous group of heritable connective tissue disorders characterized by defects in collagen synthesis [1]. While the hallmark features are joint hypermobility and skin hyperextensibility, the oral manifestations are significant. **Multiple pulp stones** (denticles) are a classic radiographic finding in EDS, particularly in the **Type VIII (Periodontal type)**. These are localized masses of dentin or calcified foci within the dental pulp. Other dental findings include shortened/malformed roots, hypoplastic enamel, and premature generalized periodontitis leading to early tooth loss. **2. Why the other options are incorrect:** * **Systemic Lupus Erythematosus (SLE):** SLE is a multisystem autoimmune disease. Its primary oral manifestations are painless oral ulcers (typically on the hard palate), xerostomia, and lichenoid lesions. It is not associated with the formation of pulp stones. * **Discoid Lupus Erythematosus (DLE):** DLE is the cutaneous form of lupus. Oral lesions in DLE are characterized by central erythema with peripheral radiating white striae (similar to Lichen Planus). It does not affect the internal calcification of the dental pulp. **3. NEET-PG High-Yield Pearls:** * **Pulp Stones Associations:** Besides EDS, multiple pulp stones are also seen in **Dentogenesis Imperfecta**, **Osteogenesis Imperfecta**, and **Calcinosis Cutis**. * **Gorlin’s Sign:** The ability to touch the tip of the nose with the tongue; seen in 50% of EDS patients due to a hypermobile lingual frenulum. * **Beighton Score:** Used to clinically assess joint hypermobility in EDS [1]. * **Vascular EDS (Type IV):** The most severe form, associated with mutations in *COL3A1*, leading to spontaneous arterial or organ rupture [1].

Q: Joint erosions are not a feature of which of the following conditions?

Systemic lupus erythematosus. The hallmark of joint involvement in **Systemic Lupus Erythematosus (SLE)** is that it is **non-erosive** [1]. While over 90% of SLE patients experience inflammatory polyarthritis, the underlying pathology involves ligamentous laxity and joint capsule weakening rather than direct destruction of bone or cartilage. This leads to **Jaccoud’s Arthropathy**, characterized by reducible deformities (like ulnar deviation or swan-neck deformity) that disappear when the hand is placed flat on a table, as the joint architecture remains intact on X-ray. **Analysis of Incorrect Options:** * **Rheumatoid Arthritis (RA):** The definitive feature of RA is marginal erosions caused by "pannus" formation (proliferating synovium) that invades bone at the joint margins where protective cartilage is absent [1]. * **Psoriatic Arthritis (PsA):** This is an erosive seronegative spondyloarthropathy [1]. It often presents with "pencil-in-cup" deformities due to extensive periarticular bone erosion and resorption. Arthritis mutilans is a deforming erosive arthritis targeting the fingers and toes that occurs in some cases of PsA [1]. * **Multicentric Reticulohistiocytosis:** A rare systemic disease where histiocytic infiltration leads to severe, rapidly progressive, and symmetric "mutilating" erosive arthritis, often involving the DIP joints. **Clinical Pearls for NEET-PG:** * **SLE vs. RA:** Both cause symmetric small joint polyarthritis, but SLE is **non-erosive** and **reducible**, whereas RA is **erosive** and leads to **fixed** deformities [1]. * **Rhupus Syndrome:** A rare overlap syndrome where a patient meets criteria for both SLE and RA; in this specific case, erosions *may* be seen. * **Radiology Tip:** If a question mentions "deformity without erosions" or "reducible ulnar deviation," always think of SLE (Jaccoud’s).

Question 1

Which of the following is FALSE about SAPHO syndrome?

Accepted Answer

The 'P' in SAPHO stands for psoriasis

Answer

Rheumatoid factor negative

Answer

HLA B-27 positive

Answer

The 'S' in SAPHO stands for synovitis

Question 2

Pulseless disease is also known as which of the following conditions?

Accepted Answer

Takayasu arteritis

Answer

Giant cell arteritis

Answer

Kawasaki disease

Answer

Polyarteritis nodosa

Question 3

A 50-year old female presents with sudden blindness. There is scalp tenderness and pain while combing her hair. Temporal artery biopsy shows intimal thickening with granulomatous inflammation. What is the diagnosis?

Accepted Answer

Giant cell arteritis

Answer

Takayasu arteritis

Answer

Polyarteritis nodosa

Answer

Microscopic polyangiitis

Question 4

Felty syndrome is characterized by all the following except?

Accepted Answer

Thrombocytopenia

Answer

Rheumatoid arthritis

Answer

Splenomegaly

Answer

Neutropenia

Question 5

A 34-year-old female presents with hypertension, pinched facies, and progressive dysphagia with a decreased tone of the lower esophageal sphincter. What is the clinical diagnosis?

Accepted Answer

Scleroderma

Answer

Hiatus hernia

Answer

Rolling hernia

Answer

Diffuse esophageal spasm

Question 6

Which is a common antibody found in Polymyositis and Dermatomyositis?

Accepted Answer

Anti-aminoacyl-tRNA synthetase

Answer

SS-7

Answer

SS-8

Answer

Anti hystidyl transfer synthetase

Question 7

All the following features are found in Sjogren syndrome, EXCEPT?

Accepted Answer

Granulomas in salivary glands

Answer

Lymphoma

Answer

Xerostomia

Answer

Xerophthalmia

Question 8

Multiple pulp stones are seen in which condition?

Accepted Answer

Ehlers Danlos syndrome

Answer

Systemic Lupus Erythematosus (SLE)

Answer

Discoid Lupus Erythematosus (DLE)

Answer

All of the above

Question 9

Joint erosions are not a feature of which of the following conditions?

Accepted Answer

Systemic lupus erythematosus

Answer

Rheumatoid arthritis

Answer

Psoriasis

Answer

Multicentric reticulohistiocytosis

Question 10

Which one of the following clinical features is not characteristic of giant cell arteritis?

Accepted Answer

Palatal paralysis

Answer

Headache is usually the first symptom

Answer

Jaw pain during chewing

Answer

Loss of visual acuity

Rheumatology and Immunology — MCQs

Rheumatology and Immunology — MCQs

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