Rheumatology and Immunology — MCQs

Rheumatology and Immunology Indian Medical PG Practice Questions and MCQs

Question 191: Which of the following is FALSE about SAPHO syndrome?

A. Rheumatoid factor negative
B. The 'P' in SAPHO stands for psoriasis (Correct Answer)
C. HLA B-27 positive
D. The 'S' in SAPHO stands for synovitis

Explanation: 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.

Question 192: Pulseless disease is also known as which of the following conditions?

A. Giant cell arteritis
B. Takayasu arteritis (Correct Answer)
C. Kawasaki disease
D. Polyarteritis nodosa

Explanation: **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).

Question 193: 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?

A. Giant cell arteritis (Correct Answer)
B. Takayasu arteritis
C. Polyarteritis nodosa
D. Microscopic polyangiitis

Explanation: ### 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].

Question 194: Felty syndrome is characterized by all the following except?

A. Rheumatoid arthritis
B. Splenomegaly
C. Neutropenia
D. Thrombocytopenia (Correct Answer)

Explanation: ### Explanation **Felty Syndrome** is a rare but severe extra-articular manifestation of long-standing, seropositive Rheumatoid Arthritis (RA). It is classically defined by a **clinical triad**: [1] 1. **Rheumatoid Arthritis:** Usually chronic, deforming, and erosive. [1] 2. **Splenomegaly:** Enlargement of the spleen is a hallmark feature. [1] 3. **Neutropenia:** An absolute neutrophil count (ANC) < 2000/mm³, leading to an increased risk of recurrent bacterial infections. #### Why Thrombocytopenia is the Correct Answer: While Felty syndrome involves the hematopoietic system, the defining hematologic abnormality is **neutropenia**, not thrombocytopenia. Although mild anemia or mild thrombocytopenia can occasionally occur due to hypersplenism, they are **not** part of the diagnostic criteria or the classic triad. Therefore, Option D is the "except" statement. #### Analysis of Other Options: * **Option A (Rheumatoid Arthritis):** This is the foundational component. Patients typically have high titers of Rheumatoid Factor (RF) and anti-CCP antibodies. [1] * **Option B (Splenomegaly):** Present in almost all cases, though the size of the spleen does not always correlate with the severity of the neutropenia. [1] * **Option C (Neutropenia):** The most critical hematologic feature, resulting from both decreased production in the bone marrow and increased peripheral destruction/sequestration. #### NEET-PG High-Yield Pearls: * **HLA Association:** Strongly associated with **HLA-DR4**. * **Risk Factors:** More common in Caucasians, males (relative to standard RA ratios), and those with long-standing disease (>10 years). [1] * **Large Granular Lymphocyte (LGL) Leukemia:** This is the primary differential diagnosis. Both present with RA, splenomegaly, and neutropenia; however, LGL leukemia is characterized by a clonal expansion of lymphocytes. * **Complications:** The most common cause of morbidity and mortality in Felty syndrome is **recurrent pulmonary and skin infections** due to neutropenia.

Question 195: 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?

A. Scleroderma (Correct Answer)
B. Hiatus hernia
C. Rolling hernia
D. Diffuse esophageal spasm

Explanation: ### Explanation The clinical presentation of **hypertension, pinched facies (beaked nose/microstomia), and esophageal dysmotility** is classic for **Systemic Sclerosis (Scleroderma)**. [1] **Why Scleroderma is correct:** Scleroderma is a multisystem connective tissue disorder characterized by excessive collagen deposition. [1] * **Pinched Facies:** Skin fibrosis leads to a "mask-like" appearance, thinning of the lips (microstomia), and a prominent, beaked nose. * **Esophageal Involvement:** This is the most common GI manifestation (80% of cases). Fibrosis of the smooth muscle in the distal two-thirds of the esophagus leads to **decreased Lower Esophageal Sphincter (LES) tone** and aperistalsis. [2] This results in severe GERD and progressive dysphagia. * **Hypertension:** Often indicates renal involvement (Scleroderma Renal Crisis), a life-threatening complication. **Why other options are incorrect:** * **Hiatus Hernia (Sliding/Rolling):** While these can cause GERD or dysphagia, they do not present with systemic features like pinched facies or hypertension. In sliding hernias, the LES tone may be low, but the characteristic facial skin changes are absent. * **Diffuse Esophageal Spasm (DES):** This presents with "corkscrew esophagus" on barium swallow and intermittent chest pain. Crucially, DES is characterized by **high-amplitude, uncoordinated contractions**, not the decreased LES tone and aperistalsis seen in Scleroderma. [2] **High-Yield Clinical Pearls for NEET-PG:** * **CREST Syndrome:** A limited form of scleroderma (Calcinosis, Raynaud’s, Esophageal dysmotility, Sclerodactyly, Telangiectasia). [2] * **Antibodies:** **Anti-Scl-70** (topoisomerase I) is specific for Diffuse Scleroderma; **Anti-centromere** is specific for Limited/CREST. * **Manometry:** Shows "low-pressure" LES, unlike Achalasia which shows "high-pressure" non-relaxing LES. [3] * **Drug of Choice for Renal Crisis:** ACE Inhibitors (Enalapril), even if creatinine is elevated.

Question 196: Which is a common antibody found in Polymyositis and Dermatomyositis?

A. SS-7
B. SS-8
C. Anti-aminoacyl-tRNA synthetase (Correct Answer)
D. Anti hystidyl transfer synthetase

Explanation: ### Explanation **Correct Option: C. Anti-aminoacyl-tRNA synthetase** **Medical Concept:** Polymyositis (PM) and Dermatomyositis (DM) are Idiopathic Inflammatory Myopathies (IIM). The most common and clinically significant group of autoantibodies in these conditions are the **Myositis-Specific Antibodies (MSAs)** [1]. Among these, the **Anti-aminoacyl-tRNA synthetase** antibodies are the most frequent. These antibodies target the enzymes responsible for attaching specific amino acids to their corresponding tRNA during protein synthesis. The most well-known antibody within this group is **Anti-Jo-1** (directed against histidyl-tRNA synthetase). Its presence defines the **Antisynthetase Syndrome**, characterized by the triad of interstitial lung disease (ILD), inflammatory arthritis, and "mechanic’s hands" [1]. **Analysis of Incorrect Options:** * **A & B (SS-7 and SS-8):** These are not recognized medical terms for antibodies in rheumatology. They appear to be distractors mimicking the nomenclature of Sjögren’s syndrome antibodies (SS-A/Ro and SS-B/La) [2]. * **D (Anti hystidyl transfer synthetase):** While "Anti-histidyl-tRNA synthetase" (Anti-Jo-1) is a specific type of anti-synthetase antibody, Option C is the broader, more accurate category that encompasses all such antibodies (including Anti-PL-7, Anti-PL-12, etc.) found in these patients. **High-Yield Clinical Pearls for NEET-PG:** * **Anti-Jo-1:** Most common MSA; strongly associated with **Interstitial Lung Disease (ILD)** [1]. * **Anti-Mi-2:** Highly specific for **Dermatomyositis**; usually carries a good prognosis and classic skin findings (Gottron papules, Heliotrope rash) [1]. * **Anti-SRP (Signal Recognition Particle):** Associated with severe, necrotizing myopathy and poor response to therapy. * **Anti-MDA5:** Associated with clinically amyopathic dermatomyositis and **rapidly progressive ILD**. * **Malignancy Risk:** Dermatomyositis has a higher association with underlying malignancy compared to Polymyositis.

Question 197: All the following features are found in Sjogren syndrome, EXCEPT?

A. Lymphoma
B. Granulomas in salivary glands (Correct Answer)
C. Xerostomia
D. Xerophthalmia

Explanation: **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).

Question 198: Multiple pulp stones are seen in which condition?

A. Ehlers Danlos syndrome (Correct Answer)
B. Systemic Lupus Erythematosus (SLE)
C. Discoid Lupus Erythematosus (DLE)
D. All of the above

Explanation: **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].

Question 199: Joint erosions are not a feature of which of the following conditions?

A. Rheumatoid arthritis
B. Psoriasis
C. Multicentric reticulohistiocytosis
D. Systemic lupus erythematosus (Correct Answer)

Explanation: 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 200: Which one of the following clinical features is not characteristic of giant cell arteritis?

A. Headache is usually the first symptom
B. Jaw pain during chewing
C. Palatal paralysis (Correct Answer)
D. Loss of visual acuity

Explanation: **Explanation:** Giant Cell Arteritis (GCA), or temporal arteritis, is a large-vessel vasculitis that primarily affects the extracranial branches of the carotid artery [2]. **Why Palatal Paralysis is the correct answer:** Palatal paralysis is not a feature of GCA. GCA typically involves the **external carotid artery branches** (temporal, maxillary, and ophthalmic arteries). Palatal paralysis would imply involvement of the vagus nerve (CN X) or its branches, which is not characteristic of this vasculitis. While GCA can rarely cause cranial nerve palsies (like Oculomotor), palatal involvement is clinically inconsistent with the disease pattern. **Analysis of Incorrect Options:** * **A. Headache:** This is the **most common** and often the first symptom (seen in ~75% of cases). It is typically new-onset, localized to the temporal region, and may be associated with scalp tenderness (e.g., pain when combing hair). * **B. Jaw Claudication:** This is the **most specific** clinical feature of GCA. It results from ischemia of the masseter muscles due to vasculitis of the maxillary artery. Pain occurs during chewing and resolves with rest. * **C. Loss of visual acuity:** This is the most dreaded complication. It is caused by **Anterior Ischemic Optic Neuropathy (AION)** due to occlusion of the posterior ciliary arteries [1]. It can lead to permanent blindness if not treated urgently with steroids. **High-Yield Clinical Pearls for NEET-PG:** * **Demographics:** Almost exclusively occurs in patients **>50 years** of age. * **Association:** Strongly associated with **Polymyalgia Rheumatica (PMR)** in 50% of cases (proximal muscle stiffness) [1]. * **Diagnosis:** Gold standard is **Temporal Artery Biopsy** (shows skip lesions and granulomatous inflammation) [1]. * **Lab:** Characteristically high **ESR (>50 mm/hr)** and CRP [1]. * **Treatment:** Start high-dose corticosteroids **immediately** if GCA is suspected to prevent visual loss; do not wait for biopsy results [1].

Practice by Chapter

Rheumatoid Arthritis

Practice Questions

Spondyloarthropathies

Practice Questions

Systemic Lupus Erythematosus

Practice Questions

Vasculitis Syndromes

Practice Questions

Scleroderma and Related Disorders

Practice Questions

Inflammatory Myopathies

Practice Questions

Crystal Arthropathies

Practice Questions

Osteoarthritis

Practice Questions

Primary Immunodeficiency Disorders

Practice Questions

Autoinflammatory Syndromes

Practice Questions

Sjögren's Syndrome

Practice Questions

Antiphospholipid Syndrome

Practice Questions

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